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Sample records for follicular lymphoma presenting

  1. Follicular Lymphoma Presenting with Leptomeningeal Disease

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    Rubens Costa; Ricardo Costa; Renata Costa

    2014-01-01

    Follicular lymphoma is generally an indolent B cell lymphoproliferative disorder of transformed follicular center B cells. Central nervous system metastasis is a very rare complication portending a very poor prognosis. We report a rare case of follicular lymphoma presenting with leptomeningeal involvement achieving a complete remission after initial therapy.

  2. Follicular Lymphoma Presenting with Leptomeningeal Disease

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    Rubens Costa

    2014-01-01

    Full Text Available Follicular lymphoma is generally an indolent B cell lymphoproliferative disorder of transformed follicular center B cells. Central nervous system metastasis is a very rare complication portending a very poor prognosis. We report a rare case of follicular lymphoma presenting with leptomeningeal involvement achieving a complete remission after initial therapy.

  3. A Rare Presentation of In Situ Mantle Cell Lymphoma and Follicular Lymphoma: A Case Report and Review of the Literature

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    Josephine Taverna

    2014-01-01

    Full Text Available A 65-year-old gentleman presented with left groin swelling over the course of two months. Physical exam revealed nontender left inguinal adenopathy, and computed tomography scans detected multiple lymph nodes in the mesenteric, aortocaval, and right common iliac regions. An excisional lymph node biopsy was performed. Pathologic evaluation demonstrated follicular center site which stained positive for PAX5, CD20, CD10, Bcl-2, Bcl-6, and mantle zone cells. These findings demonstrated CCND1 and CD5 positivity, suggesting composite lymphoma comprising follicular lymphoma (FL with in situ mantle cell lymphoma (MCLIS. FL is known as indolent non-Hodgkin lymphoma; however, the clinical significance of a coexisting MCLIS continues to be elusive, and optimal management of these patients remains largely unknown. This case illustrates the diagnostic and therapeutic challenges of composite lymphomas. This paper also discusses advances in molecular pathogenesis and lymphoma genomics which offer novel insights into these rare diseases.

  4. In situ follicular neoplasia/lymphoma: Three illustrative cases exemplifying unique disease presentations

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    Uma Sakhadeo

    2012-01-01

    Full Text Available We report three elderly patients with follicular lymphoma in situ (FLIS each highlighting a unique pattern of disease presentation and progression. The first patient had incidentally detected FLIS with peripheral blood spill and yet had an 11-year uneventful follow up. The second patient with an overt follicular lymphoma (FL developed high-grade transformation in jejunum with FLIS extensively involving the Payers patches. The third patient had a FLIS but that qualified as higher grade and was treated in spite of lack of overt FL mainly because of higher grade and patient subsequently did develop overt FL. The first case of typical FLIS confirms that peripheral blood spill does not connote poor prognosis in FLIS, the second case illustrates that FLIS may colonize mucosa-associated lymphoid tissue as part of homing in process of a disseminated FL and the third case validates the aggressive nature of high-grade FLIS.

  5. Ocular Adnexal Follicular Lymphoma

    DEFF Research Database (Denmark)

    Rasmussen, Peter K; Coupland, Sarah E; Finger, Paul T

    2014-01-01

    , and 31 (45%) had stage IIE lymphoma. Patients with disseminated lymphoma had stage IIIE (9 of 19 [47%]) and stage IV (10 of 19 [53%]) disease, whereas patients with a relapse of systemic lymphoma presented with stage IE (8 of 10 [80%]), stage IIE (1 of 10 [10%]), and stage IIIE (1 of 10 [10%]) disease...

  6. Radiation therapy of follicular lymphoma

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    Koguchi, Masahiko; Nakamura, Naoki; Tsubokura, Takuji; Gomi, Koutarou; Yamashita, Takashi [Japanese Foundation for Cancer Research, Tokyo (Japan). Hospital; Shikama, Naoto

    2001-09-01

    The follicular lymphoma, exactly, the cancer of follicular center and germinal center B lymphocytes, is reviewed on its immunological, pathological and genetic diagnoses, epidemiology, clinical symptoms, prognosis factors, therapy and assessment of therapy effects together with respective therapy of follicular small cleaved and follicular mixed small cleaved and large cell lymphoma of grade I, II; and of follicular large cell lymphoma of grade III. The therapy is essentially the radiotherapy combined with chemotherapy and others, of which effect is mainly assessed by CT. In clinical application grade II, III, irradiation of X- and electron rays and their combination is done in a fractionated manner with the maximal dose of around 35 Gy. In clinical disease grade II, III, regimen of irradiation is not fixed. In III, IV, chemotherapy and immunotherapy are major. In recurrence and malignant transformation, there is a report of large dose chemotherapy + whole body irradiation + bone marrow transplantation. (K.H.)

  7. Obinutuzumab in follicular lymphoma.

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    Martinez-Calle, N; Figueroa-Mora, R; Villar-Fernandez, S; Marcos-Jubilar, M; Panizo, C

    2016-12-01

    The CD20 marker continues to be exploited as a therapeutic target for non-Hodgkin's lymphoma. Obinutuzumab is part of a new generation of anti-CD20 monoclonal antibodies, which are synthesized using molecular engineering technology, resulting in novel target epitopes and unprecedented optimization of antibody-dependent cellular cytotoxicity and antibody-dependent cellular phagocytosis. Rituximab is the current gold standard for anti-CD20 therapy, yet despite outstanding results published over the past decade, many patients continue to relapse after anti-CD20 regimens. Obinutuzumab is slowly positioning itself in the treatment of CD20+ B-cell neoplasms. On the basis of favorable results from the phase III GADOLIN trial, obinutuzumab was recently approved by the U.S. Food and Drug Administration in combination with bendamustine followed by obinutuzumab maintenance, for the treatment of follicular lymphoma (FL) patients who relapsed or are refractory to a rituximab-containing regimen. Additional phase III trials are underway to test obinutuzumab as a first-line anti-CD20 agent in FL with good preliminary results (GALLIUM trial); thus, it is likely that obinutuzumab will soon achieve a first-line indication. It is plausible that obinutuzumab will replace rituximab as the gold standard for chemoimmunotherapy in FL, although some safety concerns still need to be resolved. This review will address the preclinical pharmacology and the main aspects of the clinical development of obinutuzumab for the treatment of FL.

  8. Entourage: the immune microenvironment following follicular lymphoma

    OpenAIRE

    2012-01-01

    In follicular lymphoma, nonmalignant immune cells are important. Follicular lymphoma depends on CD4+ cells, but CD8+ cells counteract it. We hypothesized that the presence of follicular lymphoma is associated with higher CD4+ than CD8+ cell numbers in the tumor microenvironment but not in the immune system. Using flow cytometry, pre-treatment and follow-up CD4/CD8 ratios were estimated in the bone marrow, blood and lymph nodes of untreated follicular lymphoma patients in two independent data ...

  9. Immunotherapy with rituximab in follicular lymphomas.

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    Saguna, Carmen; Mut, Ileana Delia; Lupu, Anca Roxana; Tevet, Mihaela; Bumbea, Horia; Dragan, Cornel

    2011-04-01

    Non-Hodgkin Lymphomas (NHL) represent a recent and fascinating domain of hemato-oncology, in which remarkable progress has been made. The conventional treatments of indolent lymphomas do not extend the survival rate, nor do they cure. Recent directions are centered on using several new drugs that are capable of overcoming the mechanisms that are resistant to recovery. The initiation of immunotherapy (Rituximab in 1997) seems to have changed the natural evolution of follicular lymphomas (FL). It is possible that resistance to healing in follicular lymphomas may be neutralized with Rituximab by suppressing STAT-1 positive macrophages that are present in the cellular microenvironment.Thereinafter, the re-evaluation of recent models of prognostic and therapeutic paradigmas that were used in FL became compulsory.The purpose of the paper is to compare the evolution of patients with follicular lymphoma and the period of response, according to the treatments. The study group consisted of the 71 patients diagnosed with follicular lymphoma, out of a total of 767 malignant lymphatic proliferations with B cells, for a period of 7 years (2002-2008), at the Hematology Department, Hospital Coltea, Bucharest and Hematology Department, Universitary Hospital, BucharestResults and conclusions: Combining chemotherapy with Rituximab had better results compared to the same chemotherapy, administered alone, both in induction and in case of relapse. The overall response rate in our study group was 74.7%, out of which 42.3% complete remissions. The overall response rate was 84.61% in the Rituximab group, compared to 68.88% in patients without Rituximab.

  10. Pathologic splenic rupture in a patient with follicular lymphoma

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    Aniruddha P Dayama

    2011-11-01

    Full Text Available Follicular lymphoma (FL is the most common indolent Non Hodgkin’s lymphoma (NHL . It presents primarily with widespread disease which may be asymptomatic and involves the bone marrow in around 40% of patients . Although the disease is widespread at presentation the incidence of complications such as splenic rupture which are usually seen with other aggressive lymphomas is rare

  11. Rituximab With or Without Yttrium Y-90 Ibritumomab Tiuxetan in Treating Patients With Untreated Follicular Lymphoma

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    2016-06-15

    Stage I Grade 1 Follicular Lymphoma; Stage I Grade 2 Follicular Lymphoma; Stage II Grade 1 Contiguous Follicular Lymphoma; Stage II Grade 1 Non-Contiguous Follicular Lymphoma; Stage II Grade 2 Contiguous Follicular Lymphoma; Stage II Grade 2 Non-Contiguous Follicular Lymphoma; Stage III Grade 1 Follicular Lymphoma; Stage III Grade 2 Follicular Lymphoma; Stage IV Grade 1 Follicular Lymphoma; Stage IV Grade 2 Follicular Lymphoma

  12. NOVEL DRUGS IN FOLLICULAR LYMPHOMA

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    Giuseppe Rossi

    2016-11-01

    Full Text Available Follicular lymphoma(FL is the most common indolent non-Hodgkin lymphoma and constitutes 15% to 30% of lymphoma diagnoses. The natural history of the disease is characterized by recurrent relapses and progressively shorter remissions with a median survival of 10yrs. The impossibility of a chieving a definite cure, have prompted investigations into the possible role of more effective and less toxic strategies with innovative therapeutic agents.  Recently Casulo et al demonstrated that approximately 20% of patients with FL actually relapse within 2 years after achieving remission with R-CHOP and have a poor prognosis. It is conceivable that this particularly chemoresistant population would benefit from specifically targeting the biologic and genetic factors that likely contribute to their poor prognosis. Evolving strategies for difficult to treat FL patients have recently considered  immunomodulatory agents, new monoclonal antibodies as well as drugs targeting selective intracellular pathways. The importance of targeting the microenvironment together with the malignant FL cell has been particularly underscored. We review the most promising approaches, such as the combination of anti-CD20 antibodies with immunomodulatory drugs (Lenalidomide, with mAbs directed against other surface antigens such as CD22 and CD23 (epratuzumab, lumiliximab, with immunomodulatory antibodies such as PD-1, or with inhibitors of key steps in the B-cell receptor pathway signaling such as PI3K inibithors(idelalisib, duvelisib. Another highly attractive approach is the application of the bi-specific T-cell engaging (BiTE antibody blinatumomab which targets both CD19 and CD3 antigens. Moreover, we highlight the potential of these therapies,  taking into account their toxicity. Of course we must wait for Phase III trials results to confirm the benefit of these new treatment strategies toward a new era of chemotherapy-free treatment for follicular lymphoma.

  13. Follicular lymphoma of the ocular adnexal region

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    Rasmussen, Peter Kristian; Ralfkiaer, E.; Prause, J.U.

    2015-01-01

    with ocular adnexal follicular lymphoma were identified. Fourteen (58%) of the patients were females. The median age was 63 years (range: 42–96 years). Eleven (46%) of the patients had primary ocular adnexal lymphoma, seven (29%) had an ocular adnexal lesion in conjunction with a concurrent systemic lymphoma...

  14. Metachronous presentation of small-cell rectal carcinoma on an 18F-FDG PET/CT follow-up for follicular lymphoma

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    Yousuf Qaseem, BS

    2017-09-01

    Full Text Available We present a case of a 60-year-old woman with history of follicular lymphoma in remission presenting for an 18F-fluorodeoxyglucose positron emission tomography/computed tomography for suspected recurrence. Imaging showed widespread hypermetabolic lymphadenopathy consistent with lymphoma recurrence. A 3-month 18F-fluorodeoxyglucose positron emission tomography/computed tomography follow-up after chemotherapy showed resolution of hypermetabolic lymphadenopathy but multiple new hepatic lesions and a new subtle rectal lesion. Biopsies of both hepatic and rectal lesions revealed new diagnosis of metachronous high-grade small-cell carcinoma.

  15. New drugs for follicular lymphoma.

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    Sorigue, Marc; Ribera, Josep-Maria; Motlló, Cristina; Sancho, Juan-Manuel

    2016-10-01

    Despite the improvement in prognosis since the advent of rituximab, follicular lymphoma is still incurable and remains the cause of death of most afflicted patients. With the expanding knowledge of the pathogenesis of B-cell malignancies, in the last few years a plethora of new therapies acting through a variety of mechanisms have shown promising results. This review attempts to analyze the evidence available on these new drugs, which include new monoclonal antibodies and immunoconjugates, the anti-angiogenic and immunomodulatory agent lenalidomide, the proteasome inhibitor bortezomib, inhibitors of B-cell receptor pathway enzymes, such as ibrutinib, idelalisib, duvelisib and entospletinib, BCL2 inhibitors and checkpoint inhibitors. We conclude that despite the high expectations around the new therapeutic options for patients with refractory disease, these new drugs have side effects that require caution with their use, particularly in light of the still short follow up and the lack of both randomized trials and data on combination regimens.

  16. Pathologic splenic rupture in a patient with follicular lymphoma

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    Manoranjan Mahapatra

    2011-01-01

    Full Text Available Follicular lymphoma (FL is the most common indolent Non Hodgkin’s lymphoma (NHL . It presents primarily with widespread disease which may be asymptomatic and involves the bone marrow in around 40% of patients . Although the disease is widespread at presentation the incidence of complications such as splenic rupture which are usually seen with other aggressive lymphomas is rare

  17. Skin fragility syndrome in a cat with multicentric follicular lymphoma.

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    Crosaz, Odile; Vilaplana-Grosso, Federico; Alleaume, Charline; Cordonnier, Nathalie; Bedu-Leperlier, Anne-Sophie; Marignac, Geneviève; Hubert, Blaise; Rosenberg, Dan

    2013-10-01

    An 11-year-old, spayed female domestic shorthair cat was presented for a right flank wound. On clinical examination, a single non-painful skin tear lesion with irregular edges was detected. During the examination, star-shaped cigarette paper-like skin lesions appeared spontaneously. An abdominal mass was also palpated. Feline skin fragility syndrome (FSFS) was suspected and a multicentric lymphoma was diagnosed by fine needle aspiration. The cat's condition declined and it died spontaneously. Post-mortem examination confirmed the diagnosis of lymphoma. Neoplastic lymphocytes were not observed in the skin. Histological analysis of the skin was consistent with the morphological aspects of FSFS. A possible direct link between the two conditions remains a matter of speculation, but this case report provides the first description of FSFS associated with multicentric follicular lymphoma. Thus, multicentric follicular lymphoma should be considered as a differential diagnosis in cats presenting with FSFS.

  18. FDG-PET in Follicular Lymphoma Management

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    C. Bodet-Milin

    2012-01-01

    Full Text Available 18-Fluoro-deoxyglucose positron emission tomography/computerised tomography (FDG PET/CT is commonly used in the management of patients with lymphomas and is recommended for both initial staging and response assessment after treatment in patients with diffuse large B-cell lymphoma and Hodgkin lymphoma. Despite the FDG avidity of follicular lymphoma (FL, FDG PET/CT is not yet applied in standard clinical practice for patients with FL. However, FDG PET/CT is more accurate than conventional imaging for initial staging, often prompting significant management change, and allows noninvasive characterization to guide assessment of high-grade transformation. For restaging, FDG PET/CT assists in distinguishing between scar tissue and viable tumors in residual masses and a positive PET after induction treatment would seem to predict a shorter progression-free survival.

  19. Simultaneous occurrence of follicular lymphoma and mixed-cellularity Hodgkin's lymphoma: lymph node and extranodal involvement

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    Grangeiro Maria do Patrocínio F.

    2004-01-01

    Full Text Available An unusual and well-characterised case of composite lymphoma in the spleen and lymph node is presented. The simultaneous occurrence of mixed-cellularity Hodgkin's lymphoma (HL and follicular non-Hodgkin's lymphoma (NHL was demonstrated in a 66-year-old man admitted in our Service with anaemia, hepatosplenomegaly and multiple abdominal lymph nodes. The morphological study of the spleen and lymph node of the splenic hilum showed an infiltrate composed of two distinct neoplasias. The liver was involved by NHL infiltrate and the peripancreatic lymph node exhibited HL. The Reed-Sternberg (RS cells expressed CD 15 and CD 30, whereas the NHL cells presented standard immunohistochemical features of follicular lymphoma. To our knowledge, this is the fifth case report of concurrent spleen involvement by composite lymphoma. The incidence, clinicopathological and immunohistochemical features of this rare association are discussed.

  20. A case of follicular lymphoma complicated with mesenteric panniculitis

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    Yotaro Tamai

    2009-11-01

    Full Text Available Mesenteric panniculitis (MP is a rare disease occasionally complicated with lymphoma. A 55-year old female presented with MP accompanied by malignant lymphoma. This patient was first treated for follicular lymphoma and subsequently for panniculitis. After 6 courses of R-CHOP chemotherapy, the treatment response was partial. An additional course of salvage chemotherapy led to a complete response. Since the mesenteric mass progressed simultaneously with the regression of other lymphoma lesions, we performed a biopsy of the mesenteric mass and pathologically confirmed an MP lesion without lymphoma. Subsequent high-dose chemotherapy led to CR and the MP lesion remained stable. In the present case, MP progressed with chemotherapy. We concluded that mesenteric lesions suspected of progressing or recurring should be diagnosed pathologically even if asymptomatic.

  1. FOLLICULAR LYMPHOMA: THE MANAGEMENT OF ELDERLY PATIENT

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    Alessia Castellino

    2016-12-01

    Full Text Available Follicular lymphoma (FL is the most common indolent non-Hodgkin lymphoma, typically affected mature adults and elderly, with a median age at diagnosis of 65 years. The natural history of FL appears to have been favorably impacted by the introduction of Rituximab. Randomized clinical trials have demonstrated that the addition of rituximab to standard chemotherapy induction has improved the overall survival and new strategies of chemo-immunotherapy, such as Bendamustine combined with Rituximab, showed optimal results on response and lower hematological toxicity, becoming one of the standard treatments, particularly in elderly. Moreover maintenance therapy with Rituximab demonstrated improvement of progression-free survival. Despite these exciting results, FL is still an incurable disease. It remains a critical unmet clinical need finding new prognostic factors to better identify poor outcome patients, to reduce the risk of transformation and to explore new treatment strategies, especially for patients not candidate to intensive chemotherapy regimens, such as elderly patients. Some progresses were already done with novel agents, but larger and more validated studies are needed. Elderly patients are the larger portion of patients with FL and represent a subgroup with higher treatment difficulties, because of comorbidities and smaller spectrum for treatment choice. Further studies, focused on elderly follicular lymphoma patients, with their peculiar characteristics, are needed in order to define the best tailored treatment at diagnosis and at the time of relapse in this setting.

  2. Cell of origin of transformed follicular lymphoma.

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    Kridel, Robert; Mottok, Anja; Farinha, Pedro; Ben-Neriah, Susana; Ennishi, Daisuke; Zheng, Yvonne; Chavez, Elizabeth A; Shulha, Hennady P; Tan, King; Chan, Fong Chun; Boyle, Merrill; Meissner, Barbara; Telenius, Adele; Sehn, Laurie H; Marra, Marco A; Shah, Sohrab P; Steidl, Christian; Connors, Joseph M; Scott, David W; Gascoyne, Randy D

    2015-10-29

    Follicular lymphoma (FL) is an indolent disease but transforms in 2% to 3% of patients per year into aggressive, large cell lymphoma, a critical event in the course of the disease associated with increased lymphoma-related mortality. Early transformation cannot be accurately predicted at the time of FL diagnosis and the biology of transformed FL (TFL) is poorly understood. Here, we assembled a cohort of 126 diagnostic FL specimens including 40 patients experiencing transformation (transformation for at least 5 years. In addition, we assembled an overlapping cohort of 155 TFL patients, including 114 cases for which paired samples were available, and assessed temporal changes of routinely available biomarkers, outcome after transformation, as well as molecular subtypes of TFL. We report that the expression of IRF4 is an independent predictor of early transformation (Hazard ratio, 13.3; P transformation predicts favorable prognosis. Moreover, applying the Lymph2Cx digital gene expression assay for diffuse large B-cell lymphoma (DLBCL) cell-of-origin determination to 110 patients with DLBCL-like TFL, we demonstrate that TFL is of the germinal-center B-cell-like subtype in the majority of cases (80%) but that a significant proportion of cases is of the activated B-cell-like (ABC) subtype (16%). These latter cases are commonly negative for BCL2 translocation and arise preferentially from BCL2 translocation-negative and/or IRF4-expressing FLs. Our study demonstrates the existence of molecular heterogeneity in TFL as well as its relationship to the antecedent FL.

  3. Rituximab Retreatment for Low-Tumor Burden Follicular Lymphoma

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    A summary of results from a randomized clinical trial of patients with low–tumor burden follicular lymphoma that compared maintenance therapy with rituximab versus retreatment with rituximab only when there was evidence of disease progression.

  4. Exploring Risk Factors for Follicular Lymphoma

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    Alexander J. Ambinder

    2012-01-01

    Full Text Available Follicular lymphoma (FL is an indolent malignancy of germinal center B cells with varied incidence across racial groups and geographic regions. Improvements in the classification of non-Hodgkin lymphoma subtypes provide an opportunity to explore associations between environmental exposures and FL incidence. Our paper found that aspects of Western lifestyle including sedentary lifestyle, obesity, and diets high in meat and milk are associated with an increased risk of FL. Diets rich in fruits and vegetables, polyunsaturated fatty acids, vitamin D, and certain antioxidants are inversely associated with FL risk. A medical history of Sjogren's syndrome, influenza vaccination, and heart disease may be associated with FL incidence. Associations between FL and exposure to pesticides, industrial solvents, hair dyes, and alcohol/tobacco were inconsistent. Genetic risk factors include variants at the 6p21.32 region of the MHC II locus, polymorphisms of the DNA repair gene XRCC3, and UV exposure in individuals with certain polymorphisms of the vitamin D receptor. Increasing our understanding of risk factors for FL must involve integrating epidemiological studies of genetics and exposures to allow for the examination of risk factors and interactions between genes and environment.

  5. Anti-ICOS Monoclonal Antibody MEDI-570 in Treating Patients With Relapsed or Refractory Peripheral T-cell Lymphoma Follicular Variant or Angioimmunoblastic T-cell Lymphoma

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    2017-09-28

    Follicular Variant Peripheral T-Cell Lymphoma; Grade 1 Follicular Lymphoma; Grade 2 Follicular Lymphoma; Grade 3a Follicular Lymphoma; Recurrent Angioimmunoblastic T-cell Lymphoma; Recurrent Follicular Lymphoma; Recurrent Mature T- and NK-Cell Non-Hodgkin Lymphoma; Recurrent Mycosis Fungoides; Recurrent Primary Cutaneous T-Cell Non-Hodgkin Lymphoma; Refractory Angioimmunoblastic T-cell Lymphoma; Refractory Follicular Lymphoma; Refractory Mature T-Cell and NK-Cell Non-Hodgkin Lymphoma; Stage IB Mycosis Fungoides; Stage II Mycosis Fungoides; Stage III Cutaneous T-Cell Non-Hodgkin Lymphoma; Stage III Mycosis Fungoides; Stage IV Cutaneous T-Cell Non-Hodgkin Lymphoma; Stage IV Mycosis Fungoides

  6. Follicular infundibulum tumour presenting as cutaneous horn

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    Jayaraman M

    1996-01-01

    Full Text Available Tumour of follicular infundibulum is an organoid tumour with a plate like growth attached to the epidermis with connection from the follicular epithelium. We are reporting such a case unusually presenting as cutaneous horn.

  7. Breast systemic follicular lymphoma in a man: a case report

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    La Mantia Elvira

    2012-07-01

    Full Text Available Abstract Introduction Breast involvement by non-Hodgkin lymphoma is particularly rare in men. We describe the case of a patient with a rapidly growing, painless gynecomastia-like nodule in the left breast. On ultrasonography, the nodule was suspicious for breast carcinoma. Case presentation A breast biopsy from a 54-year-old Caucasian man showed the morphoimmunophenotypical features of grade 3 follicular lymphoma. Moreover, fluorescence in situ hybridization analysis showed a t(14,18 translocation suggesting breast involvement by a systemic lymphoma rather than a primary breast lymphoma. The histological diagnosis was subsequently confirmed after nodule excision. Mediastinal and abdominal node involvement was then identified on computed tomography and positron emission tomography scans during staging examinations. Our patient was treated with chemotherapy. After three years our patient experienced a right retro-areolar relapse. He then received two further cycles of chemotherapy but developed a myeloid acute leukemia and, as a result of this, he subsequently died. Conclusions The rarity of breast lymphomas, especially in men, and the problems related to the therapeutic choices with these tumors require molecular techniques in association with classical histological diagnosis.

  8. miR-31 and miR-17-5p levels change during transformation of follicular lymphoma.

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    Thompson, Mary Ann; Edmonds, Mick D; Liang, Shan; McClintock-Treep, Sara; Wang, Xuan; Li, Shaoying; Eischen, Christine M

    2016-04-01

    The 30% of patients whose indolent follicular lymphoma transforms to aggressive diffuse large B-cell lymphoma (DLBCL) have poor survival. Reliable predictors of follicular B-cell lymphoma transformation to DLBCL are lacking, and diagnosis of those that will progress is challenging. MicroRNA, which regulates gene expression, has critical functions in the growth and progression of many cancers and contributes to the pathogenesis of lymphoma. Using 5 paired samples from patients who presented with follicular lymphoma and progressed to DLBCL, we identified specific microRNA differentially expressed between the two. Specifically, miR-17-5p levels were low in follicular lymphoma and increased as the disease transformed. In contrast, miR-31 expression was high in follicular lymphoma and decreased as the lymphoma progressed. These results were confirmed in additional unpaired cases of low-grade follicular lymphoma (n = 13) and high-grade follicular lymphoma grade 3 or DLBCL (n = 17). Loss of miR-31 expression in DLBCL was not due to deletion of the locus. Changes in miR-17-5p and miR-31 were not correlated with immunophenotype, genetics, or status of the MYC oncogene. However, increased miR-17-5p expression did significantly correlate with increased expression of p53 protein, which is indicative of mutant TP53. Two pro-proliferative genes, E2F2 and PI3KC2A, were identified as direct messenger RNA targets of miR-31, suggesting that these may contribute to follicular lymphoma transformation. Our results indicate that changes in miR-31 and miR-17-5p reflect the transformation of follicular lymphoma to an aggressive large B-cell lymphoma and may, along with their targets, be viable markers for this process.

  9. Immunoblastic follicular lymphoma: a very unusual transformation of low-grade follicular lymphoma.

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    Gheith, Shereen; Cornfield, Dennis; Chen, Weiyi; Singh-Kahlon, Pal; Ahmed, Basil

    2014-11-01

    A 73-year-old man, in clinical remission 17 years after radiation therapy for a localized low-grade follicular lymphoma (FL), developed extensive lymphadenopathy, ascites, and splenomegaly with splenic masses. Axillary lymph node biopsy showed FL composed of nodules of centrocytes side by side with nodules of immunoblasts rather than centroblasts. Immunophenotyping revealed conventional FL markers (BCL-2, BCL-6, and CD10) as well as MUM-1 in the immunoblastic component, suggesting postgerminal center differentiation. Fluorescence in situ hybridization showed t(14;18) in both centrocytic and immunoblastic components and a copy gain of BCL-6 predominantly in the immunoblastic component. Areas of centrocytic and of immunoblastic nodules were macrodissected separately and underwent molecular evaluation for immunoglobulin heavy chain gene rearrangement. Identical base-pair peaks were found, attesting to their clonal identity. This case represents a very unusual example of transformation of a low-grade FL to a nodular immunoblastic FL.

  10. Rapid Decline of Follicular Lymphoma-Associated Chylothorax after Low Dose Radiotherapy to Retroperitoneal Lymphoma Localization

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    Lien Van De Voorde

    2014-01-01

    Full Text Available Chylothorax is caused by disruption or obstruction of the thoracic duct or its tributaries that results in the leakage of chyle into the pleural space. A number of interventions have been used to treat chylothorax including the treatment of the underlying disease. Lymphoma is found in 70% of cases with nontraumatic malignant aetiology. Although patients usually have advanced lymphoma, supradiaphragmatic disease is not always present. We discuss the case of a 63-year-old woman presenting with progressive respiratory symptoms due to chylothorax. She was diagnosed with a stage IIE retroperitoneal grade 1 follicular lymphoma extending from the coeliac trunk towards the pelvic inlet. Despite thoracocentesis and medium-chain triglycerides (MCT, diet chylothorax reoccurred. After low dose radiotherapy (2×2 Gy to the abdominal lymphoma there was a marked decrease in lymphadenopathy at the coeliac trunk and a complete regression of the pleural fluid. In this case, radiotherapy was shown to be an effective nontoxic treatment option for lymphoma-associated chylothorax with long-term remission of pleural effusion.

  11. Follicular lymphoma in young adults: a clinicopathological and molecular study of 200 patients.

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    Duarte, Ivison X; Domeny-Duarte, Pollyanna; Wludarski, Sheila C L; Natkunam, Yasodha; Bacchi, Carlos E

    2013-09-01

    Follicular lymphoma is clinically heterogenous, and therefore necessitates the identification of prognostic markers to stratify risk groups and optimize clinical management. It is relatively rare in patients younger than 40 years, and the clinicopathologic characteristics and biological behavior in this age group are poorly understood. In the current study, samples from a cohort of 200 patients between 19 and 40 years were evaluated retrospectively with respect to clinical, histologic, and genetic features. These were then correlated with clinical outcome. The median age at presentation was 35 years with a slight female prepoderance (56%). Most of the cases are presented with nodal disease (90%). Concomitant follicular lymphoma and diffuse large B-cell lymphoma were observed in 7 (4%) patients. Immunohistologic studies showed the expression of CD10 (91%), BCL6 (97%), BCL2 (95%), MUM1/IRF4 (12%), MDM2 (17%), and CD23 (25%). BCL2 rearrangement was present in 74%, and BCL6 in 20%. The estimated overall survival of patients was 13 years (mean). The presence of anemia, elevated lactose dehydrogenase, bone marrow involvement, and high-risk follicular lymphoma international prognostic index correlated with adverse overall survival. Our findings revealed that follicular lymphoma in young adults demonstrate similarities with that of older adults, including the frequency of presentation at various anatomic sites, grade, and adverse prognostic factors.

  12. Rituximab, Cyclophosphamide, Bortezomib, and Dexamethasone in Treating Patients With Relapsed or Refractory Low-Grade Follicular Lymphoma, Waldenstrom Macroglobulinemia, or Mantle Cell Lymphoma

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    2016-04-13

    Extranodal Marginal Zone Lymphoma of Mucosa-Associated Lymphoid Tissue; Nodal Marginal Zone Lymphoma; Recurrent Grade 1 Follicular Lymphoma; Recurrent Grade 2 Follicular Lymphoma; Recurrent Mantle Cell Lymphoma; Recurrent Small Lymphocytic Lymphoma; Refractory Chronic Lymphocytic Leukemia; Splenic Marginal Zone Lymphoma; Waldenstrom Macroglobulinemia

  13. Magnifying Endoscopic Features of Follicular Lymphoma Involving the Stomach: A Report of Two Cases

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    Takata, Katsuyoshi; Kawano, Seiji; Fujii, Nobuharu; Kawahara, Yoshiro; Yoshino, Tadashi; Okada, Hiroyuki

    2016-01-01

    A 70-year-old woman presented with follicular lymphoma involving the stomach, duodenum, jejunum, bone, and lymph nodes. Esophagogastroduodenoscopy revealed multiple depressed lesions in the stomach. Examination with magnifying endoscopy showed branched abnormal vessels along with gastric pits, which were irregularly shaped but were preserved. The second case was a 45-year-old man diagnosed with stage II1 follicular lymphoma with duodenal, ileal, and colorectal involvement, as well as lymphadenopathy of the mesenteric lymph nodes. Esophagogastroduodenoscopy performed six years after the diagnosis revealed multiple erosions in the gastric body and angle. Magnifying endoscopic observation with narrow-band imaging showed that the gastric pits were only partially preserved and were destroyed in most of the stomach. Branched abnormal vessels were also seen. Pathological features were consistent with follicular lymphoma in both cases. The structural differences reported between the two cases appear to reflect distinct pathologies. Disappearance of gastric pits in the latter case seems to result from loss of epithelial cells, probably due to chronic inflammation. In both cases, branched abnormal vasculature was observed. These two cases suggest that magnified observations of abnormal branched microvasculature may facilitate endoscopic detection and recognition of the extent of gastric involvement in patients with follicular lymphoma.

  14. New drugs for follicular lymphoma in older adults.

    Science.gov (United States)

    Schmitt, Anna; Soubeyran, Pierre

    2014-06-01

    Follicular lymphoma is essentially a disease of the elderly, and the aging of the population in developed countries will increase patient numbers in coming years. Significant achievements have been made for treatment, but better understanding of the disease and major progress in biology now facilitate the development of many new drugs, which may have improved toxicity profiles making them appropriate for treatment of older adults. However, the increasing number of treatment possibilities, can also increase the toxicity risks, and unexpected toxicities specific to older adults may be encountered. Consequently, specific studies of older patients should be considered, using appropriate evaluation tools such as comprehensive geriatric assessment. This review will described the development of these new drugs, in the context of the treatment of older-adults with follicular lymphoma.

  15. Expression of thyroglobulin on follicular dendritic cells of thyroid mucosa-associated lymphoid tissue (MALT lymphoma

    Directory of Open Access Journals (Sweden)

    Munemasa,Mitsuru

    2009-04-01

    Full Text Available

    Reportedly, thyroid mucosa-associated lymphoid tissue (MALT lymphoma is closely associated with Hashimoto's thyroiditis. However, it remains unknown which antigen is closely associated with thyroid MALT lymphoma. We examined whether B cell response to thyroglobulin (Tg, which is a common thyroid-specific autoantigen, is related etiologically to the pathogenesis of thyroid MALT lymphoma. Expression of human Tg antigens and Cluster of differentiation (CD 35 was examined immunohistochemically in 15 cases of thyroid MALT lymphoma using paraffin-embedded, formalin-fixed tissue specimens. In all cases of thyroid MALT lymphoma, human Tg was detected immunohistochemically in the follicular epithelial cells and follicular dendritic cells (FDCs. These FDCs were positive by double immunostaining for anti-human Tg rabbit polyclonal antibody (Ab and for CD35. Results showed that the Tg, a thyroid autoantigen, had immunostained the germinal center of the thyroid MALT lymphoma. The Tg was present in the FDCs, as revealed by the staining pattern of the germinal center;this fact was confirmed by double immunostaining of anti-human Tg mouse monoclonal Ab and anti-CD35 mouse monoclonal Ab. The results of our study suggest that Tg is an autoantigen that is recognized by thyroid MALT lymphoma cells.

  16. Pediatric-type nodal follicular lymphoma: a biologically distinct lymphoma with frequent MAPK pathway mutations.

    Science.gov (United States)

    Louissaint, Abner; Schafernak, Kristian T; Geyer, Julia T; Kovach, Alexandra E; Ghandi, Mahmoud; Gratzinger, Dita; Roth, Christine G; Paxton, Christian N; Kim, Sunhee; Namgyal, Chungdak; Morin, Ryan; Morgan, Elizabeth A; Neuberg, Donna S; South, Sarah T; Harris, Marian H; Hasserjian, Robert P; Hochberg, Ephraim P; Garraway, Levi A; Harris, Nancy Lee; Weinstock, David M

    2016-08-25

    Pediatric-type nodal follicular lymphoma (PTNFL) is a variant of follicular lymphoma (FL) characterized by limited-stage presentation and invariably benign behavior despite often high-grade histological appearance. It is important to distinguish PTNFL from typical FL in order to avoid unnecessary treatment; however, this distinction relies solely on clinical and pathological criteria, which may be variably applied. To define the genetic landscape of PTNFL, we performed copy number analysis and exome and/or targeted sequencing of 26 PTNFLs (16 pediatric and 10 adult). The most commonly mutated gene in PTNFL was MAP2K1, encoding MEK1, with a mutation frequency of 43%. All MAP2K1 mutations were activating missense mutations localized to exons 2 and 3, which encode negative regulatory and catalytic domains, respectively. Missense mutations in MAPK1 (2/22) and RRAS (1/22) were identified in cases that lacked MAP2K1 mutations. The second most commonly mutated gene in PTNFL was TNFRSF14, with a mutation frequency of 29%, similar to that seen in limited-stage typical FL (P = .35). PTNFL was otherwise genomically bland and specifically lacked recurrent mutations in epigenetic modifiers (eg, CREBBP, KMT2D). Copy number aberrations affected a mean of only 0.5% of PTNFL genomes, compared with 10% of limited-stage typical FL genomes (P < .02). Importantly, the mutational profiles of PTNFLs in children and adults were highly similar. Together, these findings define PTNFL as a biologically and clinically distinct indolent lymphoma of children and adults characterized by a high prevalence of MAPK pathway mutations and a near absence of mutations in epigenetic modifiers.

  17. FDG PET/CT predictive role in follicular lymphoma

    Energy Technology Data Exchange (ETDEWEB)

    Lopci, Egesta [University Hospital S. Orsola-Malpighi Alma Mater Studiorum, Nuclear Medicine Department, Bologna (Italy); IRCCS, Humanitas (Rozzano), Nuclear Medicine Department, Milan (Italy); Zanoni, Lucia; Fonti, Cristina; Santi, Ivan; Fanti, Stefano [University Hospital S. Orsola-Malpighi Alma Mater Studiorum, Nuclear Medicine Department, Bologna (Italy); Chiti, Arturo [IRCCS, Humanitas (Rozzano), Nuclear Medicine Department, Milan (Italy); Zinzani, Pier Luigi [University Hospital S. Orsola, Department of Hematology ' ' L. Seragnoli' ' , Bologna (Italy)

    2012-05-15

    We present findings concerning {sup 18}F-fluorodeoxyglucose (FDG) positron emission tomography (PET) at end-treatment evaluation in follicular lymphoma (FL) in order to establish possible predictive factors for progression-free survival (PFS) and patient outcome. We retrospectively analysed data from 91 consecutive FL patients (M:F = 51:40, mean age 61) referred to our PET Unit at therapy completion: 38 with an indolent form (grade 1-2) and 53 with an aggressive FL (grade 3a and b) according to the World Health Organization (WHO) classification. A total of 148 FDG PET/CT scans were analysed and findings reported as positive or negative for disease. The overall response to treatment was assessed according to the revised International Workshop Criteria (IWC). The final outcome was defined as remission or disease by taking clinical, instrumental and histological data as standards of reference, with a mean follow-up period of 3 years (range 1-8). A statistical analysis was performed with respect to PFS and patient outcome for FDG PET result, tumour grading, Follicular Lymphoma International Prognostic Index (FLIPI), disease stage and number of relapses, on uni- and multivariate analyses, with p < 0.05 considered as significant. Overall patients presented a mean PFS of 35 months (range 3-86), with a relapse rate of 42%. At final outcome, remission was achieved in 67 of 91 patients (74%). Of the different predictive factors, only FDG PET result significantly correlated with patient outcome (p = 0.0002). PET/CT performance at the end of treatment was as follows: 100% sensitivity, 99% specificity, 89% positive predictive value and 100% negative predictive value. The Kaplan-Meier analysis demonstrated a statistically significant correlation with PFS for FDG PET (p < 0.0001), FLIPI score (0-1 versus {>=}2) (p = 0.0451) and number of relapses (none versus {>=}1) (p = 0.0058). These findings were confirmed at the univariate analysis, whereas at the multivariate analysis only

  18. Immunoglobulin light chain immunohistochemistry revisited, with emphasis on reactive follicular hyperplasia versus follicular lymphoma.

    Science.gov (United States)

    Weiss, Lawrence M; Loera, Sofia; Bacchi, Carlos E

    2010-05-01

    The identification of monotypic light chains is an important adjunct to the diagnosis of B-cell lymphoma, yet to reliably perform it on formalin-fixed paraffin sections is often difficult. We have evaluated a new set of monoclonal antibodies to kappa and lambda light chains that are reactive in paraffin sections. In reactive lymphoid tissues, polytypic staining was noted in greater than 95% of cases, with strong staining of plasma cells, moderate staining of the follicular dendritic cell network, and weak staining of mantle zone cells. Strong staining of the appropriate light chain was seen in each of the 7 cases of multiple myeloma. In a series of 58 cases of B-cell lymphoma, correlation between the results of immunohistochemistry and flow cytometry was obtained in 36 cases (62%), including 32 cases (21 kappa and 11 lambda) in which a single light chain was expressed. Monotypic staining was also seen in 6 additional cases (10%) in which flow cytometry was negative. Thirty of 46 cases (65%) of follicular lymphoma showed monotypic light chain expression, in contrast to 64 of 67 cases (95%) of reactive lymphoid hyperplasia, which showed polytypic light chain expression. These antibodies may provide an effective adjunct to the diagnosis of B-cell lymphoma in routine diagnostic work.

  19. Bortezomib, Rituximab, and Dexamethasone With or Without Temsirolimus in Treating Patients With Untreated or Relapsed Waldenstrom Macroglobulinemia or Relapsed or Refractory Mantle Cell or Follicular Lymphoma

    Science.gov (United States)

    2017-01-31

    Cognitive Side Effects of Cancer Therapy; Fatigue; Neurotoxicity Syndrome; Recurrent Grade 1 Follicular Lymphoma; Recurrent Grade 2 Follicular Lymphoma; Recurrent Grade 3 Follicular Lymphoma; Recurrent Mantle Cell Lymphoma; Recurrent Marginal Zone Lymphoma; Recurrent Small Lymphocytic Lymphoma; Therapy-Related Toxicity; Waldenstrom Macroglobulinemia

  20. New targets for the treatment of follicular lymphoma

    Directory of Open Access Journals (Sweden)

    Al-Katib Ayad

    2009-12-01

    Full Text Available Abstract The last two decades have witnessed striking advances in our understanding of the biological factors underlying the development of Follicular lymphoma (FL. Development of newer treatment approaches have improved the outlook for many individuals with these disorders; however, with these advances come new questions. Given the long-term survival of patients with FL, drugs with favourable side-effect profile and minimal long-term risks are desired. FL is incurable with current treatment modalities. It often runs an indolent course with multiple relapses and progressively shorter intervals of remission. The identification of new targets and development of novel targeted therapies is imperative to exploit the biology of FL while inherently preventing relapse and prolonging survival. This review summarizes the growing body of knowledge regarding novel therapeutic targets, enabling the concept of individualized targeted therapy for the treatment of FL.

  1. Immunohistochemical patterns of follicular dendritic cell meshwork and Ki-67 in small B-cell lymphomas

    Institute of Scientific and Technical Information of China (English)

    时云飞

    2013-01-01

    Objective To identify the immunohistochemical patterns of follicular dendritic cell(FDC)meshwork and Ki-67labeling index in small B-cell lymphomas(SBLs) and their significance in differential diagnosis.Methods

  2. Population Pharmacokinetics of Ofatumumab in Patients With Chronic Lymphocytic Leukemia, Follicular Lymphoma, and Rheumatoid Arthritis

    DEFF Research Database (Denmark)

    Struemper, Herbert; Sale, Mark; Patel, Bela R;

    2014-01-01

    Ofatumumab is a human monoclonal antibody directed at CD20 approved for treatment of chronic lymphocytic leukemia. The population pharmacokinetics of intravenous ofatumumab were characterized in patients with relapsed/refractory chronic lymphocytic leukemia, relapsed/refractory follicular lymphoma...

  3. Age-related differences among patients with follicular lymphoma and the importance of prognostic scoring systems : analysis from a population-based non-Hodgkin's lymphoma registry

    NARCIS (Netherlands)

    Maartense, E; le Cessie, S; Kluin-Nelemans, HC; Kluin, PM; Snijder, S; Wijermans, PW; Noordijk, EM

    2002-01-01

    Background: The influence of age on the outcome of follicular non-Hodgkin's lymphoma (FL) was studied in a population-based non-Hodgkin's lymphoma registry. Patients and methods: This study comprised 214 follicular lymphoma patients. Grade I/II was considered separately from grade III FL. The data w

  4. Classification of follicular lymphoma images: a holistic approach with symbol-based machine learning methods.

    Science.gov (United States)

    Zorman, Milan; Sánchez de la Rosa, José Luis; Dinevski, Dejan

    2011-12-01

    It is not very often to see a symbol-based machine learning approach to be used for the purpose of image classification and recognition. In this paper we will present such an approach, which we first used on the follicular lymphoma images. Lymphoma is a broad term encompassing a variety of cancers of the lymphatic system. Lymphoma is differentiated by the type of cell that multiplies and how the cancer presents itself. It is very important to get an exact diagnosis regarding lymphoma and to determine the treatments that will be most effective for the patient's condition. Our work was focused on the identification of lymphomas by finding follicles in microscopy images provided by the Laboratory of Pathology in the University Hospital of Tenerife, Spain. We divided our work in two stages: in the first stage we did image pre-processing and feature extraction, and in the second stage we used different symbolic machine learning approaches for pixel classification. Symbolic machine learning approaches are often neglected when looking for image analysis tools. They are not only known for a very appropriate knowledge representation, but also claimed to lack computational power. The results we got are very promising and show that symbolic approaches can be successful in image analysis applications.

  5. Prognostic Significance of B-cell Differentiation Genes Encoding Proteins in Diffuse Large B-cell Lymphoma and Follicular Lymphoma Grade 3

    Science.gov (United States)

    Borovečki, Ana; Korać, Petra; Nola, Marin; Ivanković, Davor; Jakšić, Branimir; Dominis, Mara

    2008-01-01

    Aim To define prognostic significance of B-cell differentiation genes encoding proteins and BCL2 and BCL6 gene abnormalities in diffuse large B-cell lymphoma and follicular lymphoma grade 3 with >75% follicular growth pattern. Methods In 53 patients with diffuse large B-cell lymphoma and 20 patients with follicular lymphoma grade 3 with >75% follicular growth pattern the following was performed: 1) determination of protein expression of BCL6, CD10, MUM1/IRF4, CD138, and BCL2 by immunohistochemistry; 2) subclassification into germinal center B-cell-like (GCB) and activated B-cell-like (ABC) groups according to the results of protein expression; 3) detection of t(14;18)(q32;q21)/IgH-BCL2 and BCL6 abnormalities by fluorescent in situ hybridization in diffuse large B-cell lymphoma and follicular lymphoma grade 3 with >75% follicular growth pattern as well as in GCB and ABC groups; and 4) assessment of the influence of the analyzed characteristics and clinical prognostic factors on overall survival. Results Isolated BCL6 expression was more frequently found in follicular lymphoma grade 3 with >75% follicular growth pattern than in diffuse large B-cell lymphoma (P = 0.030). There were no differences in BCL2 and BCL6 gene abnormalities between diffuse large B-cell lymphoma and follicular lymphoma grade 3 with >75% follicular growth pattern. Diffuse large B-cell lymphoma and follicular lymphoma grade 3 with >75% follicular growth pattern patients were equally distributed in GCB and ABC groups. t(14;18)(q32;q21) was more frequently recorded in GCB group, and t(14;18)(q32;q21) with BCL2 additional signals or only BCL2 and IgH additional signals in ABC group (P = 0.004). The GCB and ABC groups showed no difference in BCL6 gene abnormalities. There was no overall survival difference between the patients with diffuse large B-cell lymphoma or follicular lymphoma grade 3 with >75% follicular growth pattern, however, GCB group had longer overall survival than ABC group (P

  6. Autologous and allogeneic hematopoietic stem cell transplantation in follicular lymphoma.

    Science.gov (United States)

    Bhatt, V R; Armitage, J O

    2016-01-01

    High-dose chemotherapy and autologous stem cell transplantation (ASCT) improve survival in follicular lymphoma; however, relapse remains the most common cause of death. The lower risk of relapse with allogeneic SCT (alloSCT) is offset by a high transplant-related mortality (TRM). English articles indexed in the MEDLINE database were reviewed to discuss the role of graft purging, rituximab maintenance after ASCT, reduced-intensity conditioning (RIC) alloSCT, T-cell depletion, donor lymphocyte infusion (DLI) and alternate donor sources. Optimal salvage consolidation strategy may utilize ASCT following non-total body irradiation-based conditioning regimen in second remission. Rituximab maintenance after ASCT may improve molecular remission but is not yet shown to improve overall survival. RIC alloSCT permits its use in older and less-fit patients. Studies with T-cell depleted graft failed to reduce TRM despite a decline in graft-versus-host disease; however, these studies did demonstrate a therapeutic role of DLI in post-transplant relapses. In recent years, haploidentical and umbilical cord blood donors have emerged as alternative donor sources, with outcomes comparable to matched unrelated donor SCT. In the future, incorporation of novel therapeutic agents, improved risk-adapted treatment strategies, and advancement of transplant techniques may provide a better chance of survival.

  7. Follicular lymphoma (FL): Immunological tolerance theory in FL.

    Science.gov (United States)

    García-Muñoz, Ricardo; Panizo, Carlos

    2017-02-01

    The ultimate cause of follicular lymphoma (FL) remains unknown. Remarkably, almost nothing is known about immunological tolerance mechanisms that might contribute to FL development. Immunological tolerance mechanisms, like other stimuli, also induce persistent changes of B cell receptors that induce genetic instability and molecular aberrations promoting the development of a neoplasm. Using the same method as Burnet, we provide a new perspective taking advantage of the comparison of a normal linear B cell differentiation process and FL development within the framework of clonal selection theory. We propose that FL is a malignancy of cells that acquire both translocation t(14;18) and self-BCR, inducing them to proliferate and mature, resistant to negative selection. Additional genetic damage induced by non-apoptotic tolerance mechanisms, such as receptor editing, may transform a self-reactive B cell with t(14;18) into an FL. The result of tolerogenic mechanisms and genetic aberrations is the survival of FL B cell clones with similar markers and homogenous gene expression signatures despite the different stages of maturation at which the molecular damage occurs. To antagonize further growth advantage due to self-antigen recognition and chronic activation of tolerance mechanisms in the apoptosis-resistant background of FL B cells, inhibitors of BCR signaling may be promising therapeutic options.

  8. Interest of PET with F.D.G. in the follicular lymphomas; Interet de la TEP au FDG dans les lymphomes folliculaires

    Energy Technology Data Exchange (ETDEWEB)

    Albarghach, N.; Cornec, D.; Querellou, S.; Berthou, C.; Renaudineau, Y.; Pradier, O.; Cheze-Lerest, C. [CHU Morvan, 29 - Brest (France); Hatt, M.; Visvikis, D. [Inserm U650, 29 - Brest (France)

    2009-10-15

    For the follicular lymphomas, the PET is not in the systematic medical evaluation when it cannot be ignored in the evaluation of high grade non hodgkin lymphomas because it allows to make a complete extension evaluation and especially to help to define the target volume when the radiotherapy is indicated. The fixation of F.D.G. in follicular lymphomas was studied. We showed that the follicular lymphomas present in PET under the form of hypermetabolic injuries. It seems possible to consider the use of PET to help at the definition of target volumes when the radiotherapy is indicated. The intensity of fixation seems to have a predictive value for the therapy response in case of immunotherapy. The measurement of the active tumoral volume seems to have a predictive value for the response to the treatment that is worth being specified on a more important population. (N.C.)

  9. Case presentation – thyroid lymphoma

    Directory of Open Access Journals (Sweden)

    Belkisa Izić

    2011-11-01

    Full Text Available Malignant tumors of the thyroid gland account for about 1% of thenewly diagnosed malignant tumors each year, and their incidence inwomen is twice the incidence in men. According to the WHO classification (2004 thyroid tumors are divided into: carcinoma of the thyroid, adenoma and similar tumors, and other thyroid tumors which include: teratomas, angiosarcomas, paragangliomas and others, as well as primary lymphomas and plasmacytomas. Primary thyroid lymphomasare defined as lymphomas which originate in the thyroid gland. This study presents the case of a 68-year-old patient with a thyroid lymphoma, which caused compression of the airways. In the patientpresented there was reduced activity of the thyroid gland. The dominant symptoms were: breathing difficulties, hoarse voice and the enlargement of the thyroid. An ultrasound examination was performedbefore surgery on the neck, which showed a multinodular thyroid,with compromised and compressed trachea to the right and rear. Anemergency surgical procedure was performed to reduce the tumor.Pathohistological diagnosis confirmed diffuse large B cell lymphoma.The aim of the study was to present a patient with a thyroid lymphoma, who had previously not had any immunological changes to the gland,that is, she had not had any chronic lymphocyte thyroiditis, but due to the compressive syndrome it was necessary to perform an emergencysurgical procedure to reduce the tumor.

  10. Paraplegia as initial presentation of follicular thyroid carcinoma.

    Science.gov (United States)

    Haghpanah, Vahid; Abbas, Syed Imran; Mahmoodzadeh, Hossein; Shojaei, Abdolreza; Soleimani, Ali; Larijani, Bagher; Tavangar, Seyed Mohammad

    2006-03-01

    Follicular thyroid carcinoma with metastasis rarely presents with clinical picture of spinal cord compression. This report describes a 53 years old patient with follicular thyroid carcinoma who presented with paraplegia and urinary incontinence. Magnetic resonance imaging (MRI) of neck revealed a mass with destruction of C5 and extension to C6. Histopathology study demonstrated metastatic carcinoma of thyroid.

  11. Cost analysis of common treatment options for indolent follicular non-Hodgkin's lymphoma

    NARCIS (Netherlands)

    van Agthoven, M; Kramer, MHH; Sonneveld, P; van der Hem, KG; Huijgens, PC; Wijermans, PW; Kluin-Nelemans, HC; Biesma, DH; Mattijssen, [No Value; Uyl-de Groot, CA; Hagenbeek, A; Schaafsma, M.R.

    2005-01-01

    Background and Objectives. We assessed direct health care costs associated with the most commonly prescribed treatments for indolent follicular non-Hodgkin's lymphoma (FL). Design and Methods. New and previously diagnosed FL patients ( >= 18 years) known during 1997-1998 to 15 Dutch hospitals were

  12. The clinical development of obinutuzumab for the treatment of follicular lymphoma

    Directory of Open Access Journals (Sweden)

    Ma B

    2017-04-01

    Full Text Available Barbara Ma,1 Chaitra Ujjani2 1Department of Medicine, 2Lombardi Comprehensive Cancer Center, MedStar Georgetown University Hospital, Washington, DC, USA Abstract: Impressive progress has been made in recent decades for advanced-stage follicular lymphoma with the availability of anti-CD20 monoclonal antibodies, initially rituximab and more recently obinutuzumab. Obinutuzumab is a unique, third-generation, fully humanized glycoengineered IgG1 type II anti-CD20 monoclonal antibody. It has been shown to have increased antitumor activity compared to rituximab in preclinical studies, including whole-blood B-cell depletion assays, xenograft models, and primate models. This has spurred on the development of obinutuzumab through Phase I/II trials as monotherapy and in combination with chemotherapeutic agents and other targeted therapies. Its efficacy compared to rituximab and in rituximab-refractory disease has led to its continued development and eventual approval for the treatment of follicular lymphoma. Here in this review, we highlight the design and development of obinutuzumab in the treatment of advanced stage grade 1–3A follicular lymphoma and its future directions. Keywords: follicular lymphoma, obinutuzumab, anti-CD20 monoclonal antibody

  13. Methylation changes of SIRT1, KLF4, DAPK1 and SPG20 in B-lymphocytes derived from follicular and diffuse large B-cell lymphoma.

    Science.gov (United States)

    Frazzi, Raffaele; Zanetti, Eleonora; Pistoni, Mariaelena; Tamagnini, Ione; Valli, Riccardo; Braglia, Luca; Merli, Francesco

    2017-06-01

    Diffuse large-B cell lymphomas (DLBCL) and follicular lymphomas (FL) are the most represented subtypes among mature B-cell neoplasms and originate from malignant B lymphocytes. Methylation represents one of the major epigenetic mechanisms of gene regulation. Silent information regulator 1 (SIRT1) is a class III lysine-deacetylase playing several functions and considered to be a context-dependent tumor promoter. We present the quantitative methylation, gene expression and tissue distribution of SIRT1 and some key mediators related to lymphoma pathogenesis in B lymphocytes purified from biopsies of follicular hyperplasias, FL and DLBCL. SIRT1 mRNA levels are higher in FL than follicular hyperplasias and DLBCL. B cell lymphoma 6 (BCL6) positively correlates with SIRT1. SIRT1 promoter shows a methylation decrease in the order: follicular hyperplasia - FL - DLBCL. Kruppel-like factor 4 (KLF4), Death-associated protein kinase 1 (DAPK1) and Spastic Paraplegia 20 (SPG20) methylation increase significantly in FL and DLBCL compared to follicular hyperplasias. Gene expression of DAPK1 and SPG20 inversely correlates with their degree of methylation. Our findings evidence a positive correlation between SIRT1 and BCL6 expression increase in FL. SIRT1 methylation decreases in FL and DLBCL accordingly and this parallels the increase of KLF4, DAPK1 and SPG20 methylation. Copyright © 2017 The Author(s). Published by Elsevier Ltd.. All rights reserved.

  14. Diagnostic role of 18F-fluorodeoxyglucose positron emission tomography for follicular lymphoma with gastrointestinal involvement

    Institute of Scientific and Technical Information of China (English)

    Masaya Iwamuro; Hiroyuki Okada; Katsuyoshi Takata; Katsuji Shinagawa; Shigeatsu Fujiki; Junji Shiode; Atsushi Imagawa

    2012-01-01

    AIM:To investigate the capacity for 18F-fluorodeoxyglucose (18F-FDG) positron emission tomography (PET)to evaluate patients with gastrointestinal lesions of follicular lymphoma.@@METHODS:This retrospective case series consisted of 41 patients with follicular lymphoma and gastrointestinal involvement who underwent 18F-FDG-PET and endoscopic evaluations at ten different institutions between November 1996 and October 2011.Data for endoscopic,radiological,and biological examinations performed were retrospectively reviewed from clinical records.A semi-quantitative analysis of 18F-FDG uptake was performed for each involved area by calculating the maximum standardized uptake value (SUVmax).Based on the positivity of 18F-FDG uptake in the gastrointestinal lesions analyzed,patients were subdivided into two groups.To identify potential predictive factors for 18F-FDG positivity,these two groups were compared with respect to gender,age at diagnosis of lymphoma,histopathological grade,pattern of follicular dendritic cells,mitotic rate,clinical stage,soluble interleukin-2 receptor levels detected by 18F-FDG-PET,lactate dehydrogenase (LDH) levels,hemoglobin levelsbone marrow involvement,detectability of gastrointestinal lesions by computed tomography (CT) scanningand follicular lymphoma international prognostic index (FLIPI) risk.@@RESULTS:Involvement of follicular lymphoma in the stomach,duodenum,jejunum,ileum,cecum,colon,and rectum was identified in 1,34,6,3,2,3,and 6patients,respectively.No patient had esophageal involvement.In total,19/41 (46.3%) patients exhibited true-positive 18F-FDG uptake in the lesions present in their gastrointestinal tract.In contrast,false-negative 18F-FDG uptake was detected in 24 patients (58.5%),while false-positive 18F-FDG uptake was detected in 5 patients (12.2%).In the former case,2/19 patients had both 18F-FDG-positive lesions and 18F-FDG-negative lesions in the gastrointestinal tract.In patients with 18F-FDG avidity,the SUVmax value of

  15. CD7 Positive Diffuse Large B-Cell Lymphoma Arising in a Background of Follicular Lymphoma: A Case Report and Review of the Literature

    Science.gov (United States)

    Rashidi, Hooman H.

    2016-01-01

    Diffuse large B-cell lymphoma (DLBCL) is a neoplasm of large B-lymphocytes with a diffuse growth pattern. The neoplastic cells express B-cell markers such as CD20 and PAX-5 and there may be coexpression of BCL-2, BCL-6, CD10, and MUM-1. With the exception of CD5, other T-cell markers are not commonly expressed in this neoplasm. Here, we describe the first reported case of a DLBCL with abnormal expression CD7 arising in a background of follicular lymphoma in an 81-year-old male who presented with a nontender left axillary mass. Additionally, no other T-cell antigens were expressed in this B-cell lymphoma. Expression of CD7 in DLBCL is exceptionally rare and its prognostic significance is unknown. Here, we describe this rare case with review of literature of known DLBCLs with expression of T-cell antigens.

  16. Increased vascularization predicts favorable outcome in follicular lymphoma.

    NARCIS (Netherlands)

    Koster, A. de; Krieken, J.H.J.M. van; MacKenzie, M.A.; Schraders, M.; Borm, G.F.; Laak, J.A.W.M. van der; Leenders, W.P.J.; Hebeda, K.M.; Raemaekers, J.M.M.

    2005-01-01

    PURPOSE: In malignant lymphoma, angiogenesis has been associated with adverse outcome or more aggressive clinical behavior. This correlation has been established in groups of patients with a large heterogeneity regarding lymphoma subtypes and treatment regimens. The aim of this study is to investiga

  17. Germinal center reentries of BCL2-overexpressing B cells drive follicular lymphoma progression

    Science.gov (United States)

    Sungalee, Stéphanie; Mamessier, Emilie; Morgado, Ester; Grégoire, Emilie; Brohawn, Philip Z.; Morehouse, Christopher A.; Jouve, Nathalie; Monvoisin, Céline; Menard, Cédric; Debroas, Guilhaume; Faroudi, Mustapha; Mechin, Violaine; Navarro, Jean-Marc; Drevet, Charlotte; Eberle, Franziska C.; Chasson, Lionel; Baudimont, Fannie; Mancini, Stéphane J.; Tellier, Julie; Picquenot, Jean-Michel; Kelly, Rachel; Vineis, Paolo; Ruminy, Philippe; Chetaille, Bruno; Jaffe, Elaine S.; Schiff, Claudine; Hardwigsen, Jean; Tice, David A.; Higgs, Brandon W.; Tarte, Karin; Nadel, Bertrand; Roulland, Sandrine

    2014-01-01

    It has recently been demonstrated that memory B cells can reenter and reengage germinal center (GC) reactions, opening the possibility that multi-hit lymphomagenesis gradually occurs throughout life during successive immunological challenges. Here, we investigated this scenario in follicular lymphoma (FL), an indolent GC-derived malignancy. We developed a mouse model that recapitulates the FL hallmark t(14;18) translocation, which results in constitutive activation of antiapoptotic protein B cell lymphoma 2 (BCL2) in a subset of B cells, and applied a combination of molecular and immunofluorescence approaches to track normal and t(14;18)+ memory B cells in human and BCL2-overexpressing B cells in murine lymphoid tissues. BCL2-overexpressing B cells required multiple GC transits before acquiring FL-associated developmental arrest and presenting as GC B cells with constitutive activation–induced cytidine deaminase (AID) mutator activity. Moreover, multiple reentries into the GC were necessary for the progression to advanced precursor stages of FL. Together, our results demonstrate that protracted subversion of immune dynamics contributes to early dissemination and progression of t(14;18)+ precursors and shapes the systemic presentation of FL patients. PMID:25384217

  18. Characterization of intratumoral follicular helper T cells in follicular lymphoma: role in the survival of malignant B cells

    Science.gov (United States)

    Amé-Thomas, Patricia; Le Priol, Jérôme; Yssel, Hans; Caron, Gersende; Pangault, Céline; Jean, Rachel; Martin, Nadine; Marafioti, Teresa; Gaulard, Philippe; Lamy, Thierry; Fest, Thierry; Semana, Gilbert; Tarte, Karin

    2012-01-01

    Accumulating evidences indicate that the cellular and molecular microenvironment of follicular lymphoma (FL) plays a key role in both lymphomagenesis and patient outcome. Malignant FL B cells are found admixed to specific stromal and immune cell subsets, in particular CD4pos T cells displaying phenotypic features of follicular helper T cells (TFH). The goal of our study was to functionally characterize intratumoral CD4pos T cells. We showed that CXCR5hiICOShiCD4pos T cells sorted from FL biopsies comprise at least two separate cell populations with distinct genetic and functional features: i) CD25pos follicular regulatory T cells (TFR), and ii) CD25neg TFH displaying a FL-B cell supportive activity without regulatory functions. Furthermore, despite their strong similarities with tonsil-derived TFH, purified FL-derived TFH displayed a specific gene expression profile including an overexpression of several genes potentially involved directly or indirectly in lymphomagenesis, in particular TNF, LTA, IL4, or CD40LG. Interestingly, we further demonstrated that these two last signals efficiently rescued malignant B cells from spontaneous and Rituximab-induced apoptosis. Altogether, our study demonstrates that tumor-infiltrating CD4pos T cells are more heterogeneous than previously presumed, and underlines for the first time the crucial role of TFH in the complex set of cellular interactions within FL microenvironment. PMID:22015774

  19. Obinutuzumab: A Review in Rituximab-Refractory or -Relapsed Follicular Lymphoma.

    Science.gov (United States)

    Dhillon, Sohita

    2017-03-21

    Obinutuzumab (Gazyva(®), Gazyvaro(®)) is a recombinant, monoclonal, humanized and glycoengineered, type II, anti-CD20, IgG1 antibody. It has recently been granted an additional indication for the treatment of patients with follicular lymphoma who relapsed after, or are refractory to, a rituximab-containing regimen. In the primary analysis of the large, phase III GADOLIN study, induction therapy with obinutuzumab plus bendamustine followed by obinutuzumab maintenance prolonged progression-free survival (PFS) to a statistically significant extent relative to induction with bendamustine monotherapy in patients with indolent non-Hodgkin's lymphoma (iNHL). The improvement in PFS was largely driven by the subgroup of patients with follicular lymphoma, who also had prolonged overall survival (OS) in a planned updated analysis. Obinutuzumab had a generally manageable tolerability profile in these patients; mild to moderate infusion-related reactions (IRRs) were the most common treatment-emergent adverse events (AEs) and neutropenia the most common grade 3 or 4 treatment-related AEs. Although additional studies and longer-term data are needed to further assess treatment benefits with obinutuzumab, current evidence indicates that obinutuzumab is a useful treatment option for patients with rituximab-refractory or -relapsed follicular lymphoma.

  20. Treatment strategies for nodal and gastrointestinal follicular lymphoma:Current status and future development

    Institute of Scientific and Technical Information of China (English)

    Takuya; Watanabe

    2010-01-01

    In recent years,therapies for follicular lymphoma (FL) have steadily improved.A series of phase Ⅲ trials comparing the effect of rituximab with chemotherapy vs chemotherapy alone in treating FL have indicated significant improvements in progression-free survival (PFS) and overall survival.Recent studies have found that prolonged response durations and PFS were obtained with maintenance therapy using rituximab or interferon after completion of first line therapy.For patients with relapsed or refractory FL,ph...

  1. Randomized controlled trials in relapsed/refractory follicular lymphoma: a systematic review and meta-analysis.

    Science.gov (United States)

    Police, Rachel L; Trask, Peter C; Wang, Jianmin; Olivares, Robert; Khan, Shahnaz; Abbe, Adeline; Colosia, Ann; Njue, Annete; Sherril, Beth; Ruiz-Soto, Rodrigo; Kaye, James A; Hamadani, Mehdi

    2016-10-01

    This systematic literature review evaluated the clinical efficacy and safety of interventions used in relapsed/refractory follicular lymphoma. Primary efficacy outcomes were objective response rate, progression-free survival and overall survival. Safety endpoints were grade 3/4 toxicities, serious adverse events and withdrawals or deaths due to toxicity. Studies were selected if they were randomized controlled trials reporting on the efficacy or safety of treatments for relapsed or refractory follicular lymphoma, and if outcomes were reported separately from trials that included other lymphoid neoplasms. We used the Bucher method for conducting adjusted indirect comparisons within a meta-analysis. We identified 10 randomized controlled trials of treatments for relapsed/refractory follicular lymphoma. The most prominent drug investigated (alone or in combination) was rituximab. Most trials did not report median overall survival. Two trials reported median event-free survival (range, 1.2-23.2 months). Six of ten trials reported objective response rate (range, 9-93%). Meta-analysis showed only one statistically significant result: rituximab + bortezomib yielded a significantly higher objective response rate than rituximab monotherapy (relative risk, 1.28; 95% confidence interval, 1.11-1.47). Otherwise, there were no discernable differences in overall survival or progression-free survival, partly due to insufficient reporting of results in the clinical trials. The relatively small number of randomized controlled trials, few overlapping treatment arms, and variability in the randomized controlled trial features and in the endpoints studied complicate the formal comparison of therapies for relapsed/refractory follicular lymphoma. Additional well-designed randomized controlled trials are needed to fully understand the relative outcomes of older and more recently developed therapies.

  2. Diagnostic and prognostic impact of {sup 18}F-FDG PET/CT in follicular lymphoma

    Energy Technology Data Exchange (ETDEWEB)

    Le Dortz, Ludovic; Garin, Etienne [Eugene Marquis Anticancer Centre, Nuclear Medicine Department, Rennes (France); Inserm U991, Rennes (France); Guibert, Sophie de; Houot, Roch [CHU Pontchaillou, Haematological Department, Rennes (France); Bayat, Sahar; Cuggia, Marc [CHU Pontchaillou, Medical Information Department, Rennes (France); Devillers, Anne; Le Jeune, Florence; Bahri, Haifa; Barge, Marie-Luce [Eugene Marquis Anticancer Centre, Nuclear Medicine Department, Rennes (France); Rolland, Yan [Eugene Marquis Anticancer Centre, Medical Imaging Department, Rennes (France); Lamy, Thierry [CHU Pontchaillou, Haematological Department, Rennes (France); Inserm U917, Rennes (France)

    2010-12-15

    The aim of this study was to assess the usefulness of positron emission tomography/computed tomography in staging, prognosis evaluation and restaging of patients with follicular lymphoma. A retrospective study was performed on 45 patients with untreated biopsy-proven follicular lymphoma who underwent {sup 18}F-fluorodeoxyglucose PET/CT (FDG PET/CT) and CT before and after chemoimmunotherapy induction treatment (rituximab combined with cyclophosphamide, doxorubicin, vincristine and prednisone). PET/CT detected more nodal (+51%) and extranodal (+89%) lesions than CT. PET/CT modified Ann Arbor staging in eight patients (18%). Five patients (11%) initially considered as being early stage (I/II) were eventually treated as advanced stage (III/IV). In this study, an initial PET/CT prognostic score was significantly more accurate than the Follicular Lymphoma International Prognostic Index score in identifying patients with poor prognosis (i.e. patients with incomplete therapeutic response or early relapse). The accuracy of PET/CT for therapeutic response assessment was higher than that of CT (0.97 vs 0.64), especially due to its ability to identify inactive residual masses. In addition, post-treatment PET/CT was able to predict patients' outcomes. The median progression-free survival was 48 months in the PET/CT-negative group as compared with 17.2 months for the group with residual uptake (p < 10{sup -4}). FDG PET/CT is useful for staging and assessing the prognosis and therapeutic response of patients with follicular lymphoma. (orig.)

  3. Impact of treatment in long-term survival patients with follicular lymphoma: A Spanish Lymphoma Oncology Group registry

    Science.gov (United States)

    Provencio, Mariano; Sabín, Pilar; Gomez-Codina, Jose; Calvo, Virginia; Llanos, Marta; Gumá, Josep; Quero, Cristina; Blasco, Ana; Cruz, Miguel Angel; Aguiar, David; García-Arroyo, Francisco; Lavernia, Javier; Martinez, Natividad; Morales, Manuel; Saez-Cusi, Alvaro; Rodriguez, Delvys; de la Cruz, Luis; Sanchez, Jose Javier; Rueda, Antonio

    2017-01-01

    Background Follicular lymphoma is the second most common non-Hodgkin lymphoma in the United States and Europe. However, most of the prospective randomized studies have very little follow-up compared to the long natural history of the disease. The primary aim of this study was to investigate the long-term survival of our series of patients with follicular lymphoma. Patients and methods A total of 1074 patients with newly diagnosed FL were enrolled. Patients diagnosed were prospectively enrolled from 1980 to 2013. Results Median follow-up was 54.9 months and median overall survival is over 20 years in our series. We analyzed the patients who are still alive beyond 10 years from diagnosis in order to fully assess the prognostic factors that condition this group. Out of 166 patients who are still alive after more than 10 years of follow-up, 118 of them (73%) are free of evident clinical disease. Variables significantly associated with survival at 10 years were stage < II (p <0.03), age < 60 years (p <0.0001), low FLIPI (p <0.002), normal β2 microglobulin (p <0.005), no B symptoms upon diagnosis (p <0.02), Performance Status 0–1 (p <0.03) and treatment with anthracyclines and rituximab (p <0.001), or rituximab (p <0.0001). Conclusions A longer follow-up and a large series demonstrated a substantial population of patients with follicular lymphoma free of disease for more than 10 years. PMID:28493986

  4. DNA-methylome analysis in Burkitt and follicular lymphomas identifies differentially methylated regions linked to somatic mutation and transcriptional control

    Science.gov (United States)

    Betts, Matthew J.; Carrillo-de-Santa-Pau, Enrique; Doose, Gero; Gutwein, Jana; Richter, Julia; Hovestadt, Volker; Huang, Bingding; Rico, Daniel; Jühling, Frank; Kolarova, Julia; Lu, Qianhao; Otto, Christian; Wagener, Rabea; Arnolds, Judith; Burkhardt, Birgit; Claviez, Alexander; Drexler, Hans G.; Eberth, Sonja; Eils, Roland; Flicek, Paul; Haas, Siegfried; Humme, Michael; Karsch, Dennis; Kerstens, Hinrik H.D.; Klapper, Wolfram; Kreuz, Markus; Lawerenz, Chris; Lenzek, Dido; Loeffler, Markus; López, Cristina; MacLeod, Roderick A.F.; Martens, Joost H.A.; Kulis, Marta; Martín-Subero, José Ignacio; Möller, Peter; Nage, Inga; Picelli, Simone; Vater, Inga; Rohde, Marius; Rosenstiel, Philip; Rosolowski, Maciej; Russell, Robert B.; Schilhabel, Markus; Schlesner, Matthias; Stadler, Peter F.; Szczepanowski, Monika; Trümper, Lorenz; Stunnenberg, Hendrik G.; Küppers, Ralf; Ammerpohl, Ole; Lichter, Peter; Siebert, Reiner; Hoffmann, Steve; Radlwimmer, Bernhard

    2017-01-01

    In spite of both having features of germinal center B-cells, Burkitt lymphomas and follicular lymphomas are biologically and clinically quite diverse. We here performed whole genome bisulfite, genome and transcriptome sequencing from 13 IG-MYC-translocation positive Burkitt lymphoma, 9 BCL2-translocation positive follicular lymphoma and four normal germinal center B-cell samples. Comparison of Burkitt and follicular lymphoma samples revealed differential methylation of intragenic regions that strongly correlated with expression of associated genes, e.g. genes active in germinal center dark zone and light zone B-cells. Integrative pathway analyses of regions differentially methylated between Burkitt and follicular lymphoma implicated DNA methylation to cooperate with somatic mutation of sphingosine-phosphate signaling, as well as the TCF3/ID3 and SWI/SNF complexes in a large fraction of Burkitt lymphomas. Taken together, our results demonstrate a tight connection between somatic mutation, DNA methylation and transcriptional control in key B-cell pathways deregulated differentially between Burkitt and other germinal center B-cell lymphomas. PMID:26437030

  5. “Double hit” follicular lymphoma with low proliferation index: A unique case and literature review

    Directory of Open Access Journals (Sweden)

    Pardis Vafaii

    2017-03-01

    Full Text Available “Double hit” lymphomas (DHLs are aggressive B-cell lymphomas with concurrent c-MYC and BCL2 and/or BCL6 gene rearrangements. DHLs are usually classified morphologically as B-cell lymphoma, unclassifiable, with features intermediate between diffuse large B-cell lymphoma (DLBCL and Burkitt lymphoma, and less commonly as DLBCL. Follicular lymphoma (FL is characterized genetically by the presence of IGH-BCL2 rearrangement. A subset of DHLs arises from FL by the acquisition of c-MYC gene rearrangement during disease progression, but FL with concurrent IGH-BCL2 and c-MYC gene initial rearrangements is rarely reported. The few reported cases had different clinical courses, including some with indolent disease. We report a case of “double hit” low grade FL with both c-MYC and BCL2 gene rearrangements but at low proliferation rate. Unlike the usual DHLs with aggressive clinical course, our patient showed at least partial response to intense chemotherapy. Review of the literature shows a few similar cases with variable clinical course, including a few indolent cases. These patients appear to respond better with more intense chemotherapy for DHL.

  6. Treated Follicular Lymphoma, Recurrent Invasive Pneumococcal Disease, Nonresponsiveness to Vaccination, and a Unique Pneumococcus

    Directory of Open Access Journals (Sweden)

    Clare Murphy

    2012-01-01

    Full Text Available A nonneutropenic patient with treated low-grade non-Hodgkin’s (Follicular lymphoma and secondary hypogammaglobulinemia recovered from pneumococcal pneumonia and septicemia (serotype 7F; ST191 subsequent to influenza A H1N1 (2009. Both infections were potentially vaccine preventable. The patient then developed pneumococcal meningitis due to a serotype 35F pneumococcus with a unique Multilocus Sequence Type (ST7004 which was not vaccine preventable. Patient management was influenced by host predisposition to pneumococcal infection, antibiotic intolerance, and poor response to polysaccharide pneumococcal vaccine. Indirect immunofluorescence with anti-human immunoglobulin confirmed a poor or intermediate response to Pneumovax II. Prophylactic erythromycin was initiated, and immunoglobulin transfusions were also commenced as a preventive strategy. ST7004 is a single locus variant of ST1635 which has been associated with the serotype 35F capsule in England. The spi gene in ST7004, which differentiates it from ST1635, is the same as the spi gene present in ST191 which could have arisen from the first disease episode suggesting that horizontal gene transfer may have occurred between different populations of pneumococci present within the patient in an attempt to evade vaccination selection pressure.

  7. [Primary gastrointestinal follicular lymphoma of the small intestine with massive hemorrhage: a report of three cases].

    Science.gov (United States)

    Sato, Akiyasu; Tsujimura, Hideki; Sugiyama, Takahiro; Maruyama, Satoshi; Yamada, Shuhei; Ono, Keiko; Wang, Xiaofei; Sugawara, Takeaki; Ise, Mikiko; Itami, Makiko; Kumagai, Kyouya

    2016-03-01

    Primary gastrointestinal follicular lymphoma (FL) has an indolent clinical presentation and many of cases are diagnosed incidentally during routine endoscopic examinations. Herein, we present 3 cases with FL of the small intestine developed massive intestinal hemorrhage that necessitated blood transfusion. In all three patients, upper and lower endoscopic examinations failed to detect the bleeding sites. Eventually, video capsule endoscopies identified ulcerative lesions in the jejunum and biopsies using single- or double-balloon endoscopy confirmed the FL diagnosis in our three cases. The respective clinical stages according to the Lugano system were I, II-1 and II-1. PET-CT did not play a significant role in identifying the gastrointestinal lesions. Two patients received rituximab monotherapy and achieved a complete response. The other remains under observation after termination of antiplatelet drug therapy. Generally, the macroscopic appearance of multiple whitish nodules and the absence of symptoms represent the typical clinical picture of gastrointestinal FL. However, this study demonstrates that patients with ulcerative lesions may be at risk for massive bleeding. Further discussion is required to determine the optimal indications for total endoscopic examination of the small intestine.

  8. Cerebral lymphoma presenting as a leukoencephalopathy

    Science.gov (United States)

    Ayuso-Peralta, L; Orti-Pareja, M; Zurdo-Hernandez, M; Jimenez-Jimenez, F; Tejeiro-Martinez, J; Ricoy, J; de la Lama, A; Bernardo, A

    2001-01-01

    Cerebral lymphoma is infrequent in immunocompetent patients. This tumour usually appears on CT and MRI as a single lesion or as multiple lesions with mass effect and homogeneous enhancement after contrast administration. A patient is described with a cerebral lymphoma, confirmed by histopathological examination, who presented as a progressive leukoencephalopathy.

 PMID:11459903

  9. Updates from the 2016 American Society of Hematology annual meeting: practice-changing studies in untreated follicular lymphoma

    Science.gov (United States)

    Owen, C.; MacDonald, D.; Aw, A.; Christofides, A.

    2017-01-01

    The 2016 annual meeting of the American Society of Hematology took place in San Diego, California, 3–6 December. At the meeting, results from key studies on the first-line treatment of follicular lymphoma were presented. Of those studies, key oral presentations included two analyzing data from the gallium study, which evaluated the efficacy and safety of obinutuzumab plus chemotherapy (G-chemo) compared with rituximab plus chemotherapy (R-chemo), followed, in responding patients with follicular lymphoma, by obinutuzumab or rituximab maintenance; results from the sabrina study, which evaluated the efficacy and safety of subcutaneous compared with intravenous rituximab; results of a cost-effectiveness analysis of first-line treatment with bendamustine and rituximab from a Canadian perspective; and results from the SAKK 35/10 study, which evaluated the safety and efficacy of rituximab plus lenalidomide compared with rituximab monotherapy. Our meeting report describes the foregoing studies and includes interviews with the Canadian investigators, plus commentaries by those investigators about the potential impact on Canadian practice.

  10. Rituximab maintenance for 2 years in patients with high tumour burden follicular lymphoma responding to rituximab plus chemotherapy (PRIMA): a phase 3, randomised controlled trial

    DEFF Research Database (Denmark)

    Salles, Gilles; Seymour, John Francis; Offner, Fritz

    2011-01-01

    Patients with follicular lymphoma can have long survival times, but disease progression typically occurs 3-5 years after initial treatment. We assessed the potential benefit of 2 years of rituximab maintenance after first-line treatment in patients with follicular lymphoma receiving a rituximab p...

  11. Low-dose total body irradiation versus combination chemotherapy for lymphomas with follicular growth pattern.

    Science.gov (United States)

    Meerwaldt, J H; Carde, P; Burgers, J M; Monconduit, M; Thomas, J; Somers, R; Sizoo, W; Glabbeke, M V; Duez, N; de Wolf-Peeters, C

    1991-10-01

    The treatment of Non-Hodgkin's lymphomas with follicular growth pattern and advanced stage of disease remains controversial. Treatments varying from no initial treatment up to aggressive combination chemotherapy have been advocated. The EORTC Lymphoma Cooperative Group has performed a randomized prospective trial comparing short duration low dose total body irradiation (TBI) vs combination chemotherapy (CHVmP) + consolidation radiotherapy. Ninety-three patients were entered; of 84 evaluable patients, 44 received TBI and 40 CHVmP. Complete remission (CR) rates were 36%--TBI and 55%--CHVmP, but overall response rates were identical, 76 versus 69%. No significant difference in freedom from progression or survival was observed. No unexpected toxicity was seen. Although numbers are small, we cannot conclude that aggressive combination chemo-radiotherapy resulted in a better survival. Our analysis confirms that there is a constant risk of relapse. Other approaches should be explored if survival benefit is the ultimate goal in treatment of this patient population.

  12. Follicular Lymphoma Tregs Have a Distinct Transcription Profile Impacting Their Migration and Retention in the Malignant Lymph Node.

    Directory of Open Access Journals (Sweden)

    Hristina Nedelkovska

    Full Text Available We have previously shown that regulatory T cells (Tregs infiltrating follicular lymphoma lymph nodes are quantitatively and qualitatively different than those infiltrating normal and reactive nodes. To gain insight into how such Treg populations differ, we performed RNA sequence (RNAseq analyses on flow sorted Tregs from all three sources. We identify several molecules that could contribute to the observed increased suppressive capacity of follicular lymphoma nodal tregs, including upregulation of CTLA-4, IL-10, and GITR, all confirmed by protein expression. In addition, we identify, and confirm functionally, a novel mechanism by which Tregs target to and accumulate within a human tumor microenvironment, through the down regulation of S1PR1, SELL (L-selectin and CCR7, potentially resulting in greater lymph node retention. In addition we identify and confirm functionally the upregulation of the chemokine receptor CXCR5 as well as the secretion of the chemokines CXCL13 and IL-16 demonstrating the unique ability of the follicular derived Tregs to localize and accumulate within not only the malignant lymph node, but also localize and accumulate within the malignant B cell follicle itself. Such findings offer significant new insights into how follicular lymphoma nodal Tregs may contribute to the biology of follicular lymphoma and identify several novel therapeutic targets.

  13. Combined assay of surface immunoglobulin intensity and mouse rosettes. A practical parameter in the differential diagnosis of small lymphocytic and follicular center cell lymphomas.

    Science.gov (United States)

    Batata, A; Shen, B

    1993-03-01

    Cell suspensions from the lymph nodes of small lymphocytic lymphoma (n = 94) and nodular and diffuse follicular center cell lymphomas (n = 330) were analyzed to evaluate the diagnostic significance of the surface immunoglobulin (SIg) intensity and mouse rosette assay (MR). In small lymphocytic lymphoma, SIg was monoclonal in 65 cases (69.15%), with weak fluorescence in 59 (90.77%). It was not detected in 29 cases (30.85%). The MR findings were positive in 68 cases (72.34%) and negative in 26 (27.66%). The combined results of these two assays showed the following: weak SIg/MR+, 35 (37.23%); weak SIg/MR-, 24 (25.53%); strong SIg/MR+, 6 (6.38%); strong SIg/MR-, 0; undetected SIg/MR+, 27 (28.72%); and undetected SIg/MR-, 2 (2.13%). By performing the assays for these two markers and accepting weak SIg/MR+, weak SIg/MR-, strong SIg/MR+, or undetected SIg/MR+ as sufficient for diagnosis, 92 cases (97.87%) were diagnosed. In diffuse follicular center cell lymphomas, SIg was monoclonal in 287 cases (86.97%), with strong fluorescence in 258 (89.9%) and weak fluorescence in 29 (10.1%). It was not detected in 43 cases (13.03%). The MR results were positive in 34 cases (10.3%) and negative in 296 (89.7%). The combined findings of these two assays showed that strong SIg/MR- was present in 244 cases (73.94%). The diagnostic value of the combined assay in the differential diagnosis between small lymphocytic lymphoma and diffuse follicular center cell lymphomas was proved using five statistical parameters.

  14. Computer-assisted quantification of CD3+ T cells in follicular lymphoma.

    Science.gov (United States)

    Abas, Fazly S; Shana'ah, Arwa; Christian, Beth; Hasserjian, Robert; Louissaint, Abner; Pennell, Michael; Sahiner, Berkman; Chen, Weijie; Niazi, Muhammad Khalid Khan; Lozanski, Gerard; Gurcan, Metin

    2017-01-22

    The advance of high resolution digital scans of pathology slides allowed development of computer based image analysis algorithms that may help pathologists in IHC stains quantification. While very promising, these methods require further refinement before they are implemented in routine clinical setting. Particularly critical is to evaluate algorithm performance in a setting similar to current clinical practice. In this article, we present a pilot study that evaluates the use of a computerized cell quantification method in the clinical estimation of CD3 positive (CD3+) T cells in follicular lymphoma (FL). Our goal is to demonstrate the degree to which computerized quantification is comparable to the practice of estimation by a panel of expert pathologists. The computerized quantification method uses entropy based histogram thresholding to separate brown (CD3+) and blue (CD3-) regions after a color space transformation. A panel of four board-certified hematopathologists evaluated a database of 20 FL images using two different reading methods: visual estimation and manual marking of each CD3+ cell in the images. These image data and the readings provided a reference standard and the range of variability among readers. Sensitivity and specificity measures of the computer's segmentation of CD3+ and CD- T cell are recorded. For all four pathologists, mean sensitivity and specificity measures are 90.97 and 88.38%, respectively. The computerized quantification method agrees more with the manual cell marking as compared to the visual estimations. Statistical comparison between the computerized quantification method and the pathologist readings demonstrated good agreement with correlation coefficient values of 0.81 and 0.96 in terms of Lin's concordance correlation and Spearman's correlation coefficient, respectively. These values are higher than most of those calculated among the pathologists. In the future, the computerized quantification method may be used to investigate

  15. Anti-tumor activity of obinutuzumab and rituximab in a follicular lymphoma 3D model.

    Science.gov (United States)

    Decaup, E; Jean, C; Laurent, C; Gravelle, P; Fruchon, S; Capilla, F; Marrot, A; Al Saati, T; Frenois, F-X; Laurent, G; Klein, C; Varoqueaux, N; Savina, A; Fournié, J-J; Bezombes, C

    2013-08-09

    Follicular lymphomas (FLs) account for 35-40% of all adult lymphomas. Treatment typically involves chemotherapy combined with the anti-CD20 monoclonal antibody (MAb) rituximab (RTX). The development of the type II anti-CD20 MAb obinutuzumab (GA101) aims to further improve treatment. Here, using FL cells we show that RTX and GA101 display a similar activity on RL cells cultured in 2D. However, 2D culture cannot mimic tumor spatial organization and conventional 2D models may not reflect the effects of antibodies as they occur in vivo. Thus, we created a non-Hodgkin's lymphoma (NHL) 3D culture system, termed multicellular aggregates of lymphoma cells (MALC), and used it to compare RTX and GA101 activity. Our results show that both antibodies display greater activity towards FL cells in 3D culture compared with 2D culture. Moreover, we observed that in the 3D model GA101 was more effective than RTX both in inhibiting MALC growth through induction of (lysosomal) cell death and senescence and in inhibiting intracellular signaling pathways, such as mammalian target of rapamycin, Akt, PLCgamma (Phospholipase C gamma) and Syk. Altogether, our study demonstrates that spatial organization strongly influences the response to antibody treatment, supporting the use of 3D models for the testing of therapeutic agents in NHL.

  16. FOXP3-stained image analysis for follicular lymphoma: optimal adaptive thresholding with maximal nucleus coverage

    Science.gov (United States)

    Senaras, C.; Pennell, M.; Chen, W.; Sahiner, B.; Shana'ah, A.; Louissaint, A.; Hasserjian, R. P.; Lozanski, G.; Gurcan, M. N.

    2017-03-01

    Immunohistochemical detection of FOXP3 antigen is a usable marker for detection of regulatory T lymphocytes (TR) in formalin fixed and paraffin embedded sections of different types of tumor tissue. TR plays a major role in homeostasis of normal immune systems where they prevent auto reactivity of the immune system towards the host. This beneficial effect of TR is frequently "hijacked" by malignant cells where tumor-infiltrating regulatory T cells are recruited by the malignant nuclei to inhibit the beneficial immune response of the host against the tumor cells. In the majority of human solid tumors, an increased number of tumor-infiltrating FOXP3 positive TR is associated with worse outcome. However, in follicular lymphoma (FL) the impact of the number and distribution of TR on the outcome still remains controversial. In this study, we present a novel method to detect and enumerate nuclei from FOXP3 stained images of FL biopsies. The proposed method defines a new adaptive thresholding procedure, namely the optimal adaptive thresholding (OAT) method, which aims to minimize under-segmented and over-segmented nuclei for coarse segmentation. Next, we integrate a parameter free elliptical arc and line segment detector (ELSD) as additional information to refine segmentation results and to split most of the merged nuclei. Finally, we utilize a state-of-the-art super-pixel method, Simple Linear Iterative Clustering (SLIC) to split the rest of the merged nuclei. Our dataset consists of 13 region-ofinterest images containing 769 negative and 88 positive nuclei. Three expert pathologists evaluated the method and reported sensitivity values in detecting negative and positive nuclei ranging from 83-100% and 90-95%, and precision values of 98-100% and 99-100%, respectively. The proposed solution can be used to investigate the impact of FOXP3 positive nuclei on the outcome and prognosis in FL.

  17. Myeloid cell nuclear differentiation antigen is expressed in a subset of marginal zone lymphomas and is useful in the differential diagnosis with follicular lymphoma.

    Science.gov (United States)

    Metcalf, Ryan A; Monabati, Ahmad; Vyas, Monika; Roncador, Giovanna; Gualco, Gabriela; Bacchi, Carlos E; Younes, Sheren F; Natkunam, Yasodha; Freud, Aharon G

    2014-08-01

    The diagnosis of marginal zone lymphomas (MZL) is challenged by the lack of specific markers that distinguish them from other low-grade non-Hodgkin B-cell lymphomas. Myeloid cell nuclear differentiation antigen (MNDA) is a nuclear protein that labels myelomonocytic cells as well as B lymphocytes that localize to the marginal zone areas of splenic white pulp. We evaluated MNDA expression in a large series of B-cell lymphomas to assess the sensitivity and specificity of this antigen for the characterization of MZL. A total of 440 tissue sections containing extramedullary B-cell lymphomas and 216 bone marrow biopsies containing atypical or neoplastic lymphoid infiltrates were stained for MNDA by immunohistochemistry. Among the extramedullary lymphoma cases, approximately 67% of nodal MZL, 61% of extranodal MZL, and 24% of splenic MZL expressed MNDA. MNDA was also infrequently expressed in other B-cell neoplasms including mantle cell lymphoma (6%), chronic lymphocytic leukemia/small lymphocytic lymphoma (13%), follicular lymphoma (FL) (4%), lymphoplasmacytic lymphoma (25%), and diffuse large B-cell lymphoma (3%). In contrast, MNDA was only expressed in 2.3% of all bone marrow biopsies involved by lymphoid infiltrates, including 2 cases of FL and one case of MZL. Collectively, these data support the inclusion of MNDA in the diagnostic evaluation of extramedullary B-cell lymphomas, particularly those in which the differential diagnosis is between low-grade FL and MZL.

  18. Collision tumor consisting of primary follicular lymphoma and adenocarcinoma in the cecum: A case report and literature review

    Science.gov (United States)

    KUS, TULAY; AKTAS, GOKMEN; KALENDER, MEHMET EMIN; SARI, IBRAHIM; ULKER, ESRA; CAMCI, CELALETDIN

    2016-01-01

    The present study reports the case of a collision tumor consisting of follicular lymphoma (FL) and adenocarcinoma in the cecum of a 73-year-old man. To the best of our knowledge, the present study is the 11th case of a collision tumor consisting of colon adenocarcinoma and lymphoma to be reported in the literature, and the first case of cecum adenocarcinoma with low grade FL in the same segment of the cecum and the same regional lymph node to be reported. The present study reviewed the literature to determine treatment options for patients with collision tumors. The present patient was administered with adjuvant chemotherapy for T3N1M0 colon cancer following surgery, due to the dominance of colon adenocarcinoma in the collision tumor. Following the completion of treatment, progression of the untreated FL was observed. In the literature, patients with collision tumors are administered with chemotherapy for stage IV FL, and following the completion of treatment patients have presented with a recurrence of early stage colon adenocarcinoma. The recommended treatment for collision tumors is dependent on the dominant tumor; however, the treatment options for collision tumors in the literature appeared to exacerbate the other tumor. The characteristics of the tumors altered following chemotherapy, and immunological alterations in the tumors due to chemotherapy appear to have contributed to the exacerbation of the tumors. Therefore, patients with early-stage tumors should be considered at risk of recurrence of other malignancies, which are present in collision tumors. PMID:27073555

  19. Validation, revision and extension of the follicular lymphoma international prognostic index (FLIPI) in a population-based setting

    NARCIS (Netherlands)

    S.A.M. van de Schans (Saskia); E.W. Steyerberg (Ewout); M.R. Nijziel (Marten); G.J.M. Creemers (Geert-Jan); M.L.G. Janssen-Heijnen (Maryska); D.J. van Spronsen (Dick Johan)

    2009-01-01

    textabstractBackground: The aim of this study was to validate the Follicular Lymphoma International Prognostic Index (FLIPI) in a population-based cohort and to study the relevance of revision and extension of the FLIPI. Patients and methods: Data of 353 unselected patients, 1993-2002, in the

  20. Follicular lymphoma (in situ) pattern in the bone marrow: does it indicate an early stage in disease evolution?

    Science.gov (United States)

    Alobeid, Bachir; Mears, John Gregory; Bhagat, Govind

    2015-01-01

    Key Clinical Message Bone marrow involvement by an isolated interstitial lymphoid aggregate exhibiting the pattern and phenotype described for follicular lymphoma in situ (FLIS) has not been reported before. The detection of clinically silent FL in this case highlights the necessity of complete staging workup when such lesions are encountered in biopsies. PMID:26185645

  1. Rapid decline of follicular lymphoma-associated chylothorax after low dose radiotherapy to retroperitoneal lymphoma localization

    NARCIS (Netherlands)

    Van De Voorde, Lien; Vanneste, Ben; Borger, Jacques; Troost, Esther G C; Werner, Philo

    2014-01-01

    Chylothorax is caused by disruption or obstruction of the thoracic duct or its tributaries that results in the leakage of chyle into the pleural space. A number of interventions have been used to treat chylothorax including the treatment of the underlying disease. Lymphoma is found in 70% of cases w

  2. Follicular thyroid carcinoma presenting as bilateral cheek masses.

    Science.gov (United States)

    Kim, Dong-Wook; Hah, J Hun; An, Soo-Youn; Chang, Hak; Kim, Kwang Hyun

    2013-03-01

    Mandibular metastasis of thyroid carcinoma is extremely rare. We present the case of a 46-year-old woman who had bilateral huge cheek masses that had grown rapidly over several years. Intra-oral mucosal tissue biopsy and imaging work-up including computed tomography scan and magnetic resonance imaging were performed and the initial diagnosis was presumed to be central giant cell granuloma. Incidentally detected thyroid lesions were studied with ultra-sonography guided fine needle aspiration and diagnosed as simple benign nodules. Due to continuous oral bleeding and the locally destructive feature of the lesions, we decided to excise the mass surgically. To avoid functional deficit, a stepwise approach was performed: Firstly, the larger left mass was excised and the mandible was reconstructed with a fibular free flap. The final pathologic diagnosis was follicular thyroid cancer. Postoperative I-131 thyroid scan and whole body positron-emissions-tomography were performed. Right side mass was revealed as a thyroid malignancy. Multiple bony metastases were detected. Since further radioactive iodine therapy was required, additional total thyroidectomy and right side mandibulectomy with fibular free flap reconstruction was performed. The patient also underwent high dose radioactive iodine therapy and palliative extra-beam radiotherapy for the metastatic lumbar lesion. Follicular thyroid carcinoma should be considered as a differential diagnosis for mandibular mass lesions.

  3. Clinical presentation and staging of Hodgkin lymphoma

    DEFF Research Database (Denmark)

    Gallamini, Andrea; Hutchings, Martin; Ramadan, Safaa

    2016-01-01

    . The main body of the review will be dedicated to the recently published guidelines for lymphoma staging (including HL) agreed by the experts during the 12th International Congress for Malignant Lymphoma in Lugano. The recommendations of the panel on how to integrate flurodeoxyglucose positron emission......, sometimes HL is a subtle disease, difficult to diagnose for the paucity of symptoms, the absence of physical findings, or for concomitant immunologic disorders: a compete overview of the common and rare patterns of HL clinical presentation will be also offered. The future perspective of PET scan use...

  4. UPDATE ON THE ROLE OF AUTOLOGOUS HEMATOPOIETIC STEM CELL TRANSPLANT IN FOLLICULAR LYMPHOMAS

    Directory of Open Access Journals (Sweden)

    Mónica Cabrero

    2012-11-01

    Full Text Available Follicular lymphoma (FL remains incurable despite advances in new strategies of treatment, including monoclonal antibodies (MoAb. Except for early stages, FL is characterized by responses to treatments and systematic relapses. The main objective in this disease is to achieve a better progression free survival (PFS and to increase overall survival (OS, mainly in young patients. In order to improve the results of conventional chemotherapy, autologous stem cell transplant (ASCT is a feasible treatment in these patients. In this moment, ASCT is not recommended as first line treatment, except for transformed FL, but is a good strategy as salvage therapy with an improved PFS and OS. New drugs have been introduced to enhance responses of ASCT, but nowadays they are not part of conventional conditioning regimen.

  5. UPDATE ON THE ROLE OF AUTOLOGOUS HEMATOPOIETIC STEM CELL TRANSPLANT IN FOLLICULAR LYMPHOMAS

    Directory of Open Access Journals (Sweden)

    Mónica Cabrero

    2012-01-01

    Full Text Available

    Follicular lymphoma (FL remains incurable despite advances in new strategies of treatment, including monoclonal antibodies (MoAb. Except for early stages, FL is characterized by responses to treatments and systematic relapses. The main objective in this disease is to achieve a better progression free survival (PFS and to increase overall survival (OS, mainly in young patients. In order to improve the results of conventional chemotherapy, autologous stem cell transplant (ASCT is a feasible treatment in these patients. In this moment, ASCT is not recommended as first line treatment, except for transformed FL, but is a good strategy as salvage therapy with an improved PFS and OS. New drugs have been introduced to enhance responses of ASCT, but nowadays they are not part of conventional conditioning regimen.

  6. Update on the Role of Autologous Hematopoietic Stem Cell Transplantation in Follicular Lymphoma

    Science.gov (United States)

    Cabrero, Mónica; Redondo, Alba; Martin, Alejandro; Caballero, Dolores

    2012-01-01

    Follicular lymphoma (FL) remains incurable despite advances in new strategies of treatment, including monoclonal antibodies (MoAb). Except for early stages, FL is characterized by responses to treatments and systematic relapses. The main objective in this disease is to achieve a better progression free survival (PFS) and to increase overall survival (OS), mainly in young patients. In order to improve the results of conventional chemotherapy, autologous stem cell transplant (ASCT) is a feasible treatment in these patients. In this moment, ASCT is not recommended as first line treatment, except for transformed FL, but is a good strategy as salvage therapy with an improved PFS and OS. New drugs have been introduced to enhance responses of ASCT, but nowadays they are not part of conventional conditioning regimen. PMID:23205262

  7. Critical appraisal of rituximab in the maintenance treatment of advanced follicular lymphoma

    Directory of Open Access Journals (Sweden)

    Aguiar-Bujanda D

    2015-10-01

    Full Text Available David Aguiar-Bujanda, María Jesús Blanco-Sánchez, María Hernández-Sosa, Saray Galván-Ruíz, Samuel Hernández-Sarmiento Department of Medical Oncology, Hospital Universitario de Gran Canaria Doctor Negrín, Las Palmas de Gran Canaria, Spain Abstract: Rituximab is an IgG1, chimeric monoclonal antibody specifically designed to recognize the CD20 antigen expressed on the surface of normal and malignant B-lymphocytes, from the B-cell precursor to the mature B-cells of the germinal center, and by most neoplasms derived from B-cells. After 2 decades of use, rituximab is firmly positioned in the treatment of follicular lymphoma (FL, both in the front line and in the relapsing disease, improving previous results by including it in classical chemotherapy regimens. However, the pharmacology of rituximab continues to generate controversial issues especially regarding the mechanisms of action in vivo. The contribution of rituximab as a maintenance treatment in FL has been significant progress in the management of this disease without an increase in side effects or a decrease in the quality of life of patients. With the widespread use of rituximab, there are new security alerts and side effects not previously detected in the pivotal trials that clinicians should learn to recognize and manage. In this article, we will review the pharmacokinetics and pharmacodynamics of rituximab, the management issues in the treatment of advanced FL focusing on maintenance rituximab, its long-term efficacy and safety profile, and its effect on the quality of life. Keywords: follicular lymphoma, long-term efficacy, maintenance, rituximab, toxicity

  8. A human follicular lymphoma B cell line hypermutates its functional immunoglobulin genes in vitro.

    Science.gov (United States)

    Wu, H; Pelkonen, E; Knuutila, S; Kaartinen, M

    1995-12-01

    The functional immunoglobulin (Ig) genes of B lymphocytes undergo somatic mutations during immune responses. These mutations modify the antigen binding site of the immunoglobulins, thereby enhancing the average affinity of the antibodies produced. The molecular mechanism underlying these B cell hypermutations remains unresolved, partly because it is difficult to grow normal B cells in long-term cell cultures and because there is no suitable transformed or malignant B cell line which generates mutations in its immunoglobulin genes in vitro. Here, we show that the recently established follicular lymphoma line HF-1.3.4 generates somatic hypermutations in vitro at a high frequency of 0.7 x 10(-6) mutations per base pair per generation in standard cell cultures (RPMI 1640 + 5% fetal calf serum). This shows for the first time that B cell hypermutation can occur without T cells or T cell factors. The mutation frequency increased approximately tenfold to 1 x 10(-5) mutations/base pair/generation with B cell-specific growth factors (interleukins-2 and -4 and three antibodies stimulatory to HF-1.3.4 cells). This HF-1.3.4 lymphoma line may help to elucidate the molecular mechanism of Ig gene hypermutation.

  9. PET/CT before autologous stem cell transplantation predicts outcome in refractory/relapsed follicular lymphoma

    Energy Technology Data Exchange (ETDEWEB)

    Alcantara, Marion; Tilly, Herve [Universite de Rouen, Service d' Hematologie, Centre Henri Becquerel, Rouen (France); Dupuis, Jehan; Haioun, Corinne [CHU Henri Mondor et Universite Paris-Est, Assistance Publique - Hopitaux de Paris, Unite Hemopathies Lymphoides, Marechal de Lattre de Tassigny (France); Mareschal, Sylvain; Dubois, Sydney [Centre Henri Becquerel, IRIB, Unite Inserm U918, Rouen (France); Julian, Anne [CHU Purpan, Service de Medecine Nucleaire, Toulouse (France); Cottereau, Anne Segolene; Becker, Stephanie [Centre Henri Becquerel, Service de Medecine Nucleaire, Rouen (France); Oberic, Lucie; Huynh, Anne; Laurent, Guy; Ysebaert, Loic [IUCT-Oncopole, Departement d' Hematologie, Toulouse (France); Meignan, Michel [CHU Henri-Mondor, Service de Medecine Nucleaire, Paris (France)

    2014-09-20

    Salvage of young patients with follicular lymphoma (FL) after R-CHOP includes salvage immunochemotherapy followed by autologous stem cell transplantation (ASCT). Previous studies dealing with relapsed Hodgkin lymphoma have shown the prognostic value of PET/CT prior to ASCT. We retrospectively analysed 59 patients with refractory/relapsed FL after first-line R-CHOP who were chemosensitive (as evaluated by CT) to the salvage treatment and who proceeded to ASCT. The role of PET/CT in this setting to define chemosensitivity is not definitely established. So we focused on the prognostic value of PET/CT performed after salvage treatment, before ASCT. The estimated 3-year progression-free survival (PFS) and overall survival were 63.1 % (50.9-78.3 %) and 90.5 % (82.8 - 98.8 %), respectively, and did not differ significantly according to their Follicular Lymphoma International Prognostic Index at relapse, conditioning regimen, or type of salvage. PFS was significantly lower in PET/CT-positive patients, according to the International Harmonization Project revised response criteria, with a 3-year PFS of 45.5 % (26.6 - 77.8 %) versus 72.6 % (58.5 - 90.0 %; p = 0.039). To better refine prognosis, we applied two types of thresholds: a Deauville five-point scale positive threshold of ≥3 (3-year PFS of 74.9 %, range 61.0 - 92.1 % %, versus 42.8 %, range 24.7 - 74.4 %; p = 0.02), and a ≥70 % ∇SUV{sub max} threshold between presalvage and pre-ASCT PET/CT (3-year PFS of 72.4 %, range 57.5 - 91.3 % versus 13.3 %, 2.2 - 81.7 %; p < 10{sup -3}). The PET/CT findings before ASCT were independently correlated with PFS in our series. PET/CT negativity before ASCT is a desirable and achievable goal in the management of chemosensitive FL relapsing after first-line R-CHOP. (orig.)

  10. Higher World Health Organization grades of follicular lymphoma correlate with better outcome in two Nordic Lymphoma Group trials of rituximab without chemotherapy

    DEFF Research Database (Denmark)

    Wahlin, Björn Engelbrekt; Sundström, Christer; Sander, Birgitta

    2014-01-01

    Abstract A common treatment for follicular lymphoma is rituximab monotherapy. To identify patients for whom this regimen is adequate as first-line therapy, we applied the World Health Organization (WHO) classification for grading follicular lymphoma in a prospective central pathology review...... of the biopsies of previously untreated patients in two randomized trials of rituximab without chemotherapy. In the first trial (n₁ = 53), higher WHO grades correlated with longer time to next treatment, independently of clinical prognostic factors (p = 0.030); the finding was replicated in the second trial (n₂...... = 221; p = 0.019). Higher grades were associated with better treatment responses (p = 0.018). Furthermore, also grades externally confirmed by independent local pathologists correlated with time to next treatment (p = 0.048). Flow cytometry in a separate patient series showed that the intensity of CD20...

  11. Update on the rational use of (90Y-ibritumomab tiuxetan in the treatment of follicular lymphoma

    Directory of Open Access Journals (Sweden)

    Martina Lehnert

    2009-07-01

    Full Text Available Martina Lehnert, Heinz Ludwig, Niklas Zojer 1st Department of Medicine, Center for Oncology and Hematology, Wilhelminenspital, Vienna, AustriaAbstract: The development of radiolabeled antibodies against CD20 has facilitated targeted treatment of follicular lymphoma (FL. By using 90Y-ibritumomab tiuxetan (Zevalin®, a radionuclide (yttrium-90, linked by the chelator tiuxetan to the antibody ibritumomab is brought into the vicinity of lymphoma cells. By the so-called cross-fire effect, this beta emitter has the capacity to destroy not only the lymphoma cells having bound the antibody, but also neighboring lymphoma cells. Currently this antibody is licensed in the European Union for use in relapsed or refractory FL. It is anticipated that this drug will also be approved for use as consolidation therapy after successful first-line treatment. Here we first will review the published literature supporting the use of 90Y-ibritumomab tiuxetan in the aforementioned indications and emerging data showing applicability of ibritumomab tiuxetan as sole first-line therapy for FL, as well as in the transplant setting. Possible strategies of incorporating ibritumomab tiuxetan into the treatment algorithm of FL are discussed.Keywords: follicular lymphoma, 90Y-ibritumomab tiuxetan

  12. Cutaneous presentation of Double Hit Lymphoma

    Directory of Open Access Journals (Sweden)

    Yousef Khelfa MD, FACP

    2016-04-01

    Full Text Available Diffuse large B-cell lymphoma (DLBCL is the most common type of non-Hodgkin lymphoma (NHL, representing approximately 25% of diagnosed NHL. DLBCL is heterogeneous disease both clinically and genetically. The 3 most common chromosomal translocations in DLBCL involve the oncogenes BCL2, BCL6, and MYC. Double hit (DH DLBCL is an aggressive form in which MYC rearrangement is associated with either BCL2 or BCL6 rearrangement. Patients typically present with a rapidly growing mass, often with B symptoms. Extranodal disease is often present. Though there is a paucity of prospective trials in this subtype, double hit lymphoma (DHL has been linked to very poor outcomes when patients are treated with standard R-CHOP. There is, therefore, a lack of consensus regarding the standard treatment for DHL. Several retrospective analyses have been conducted to help guide treatment of this disease. These suggest that DA EPOCH-R may be the most promising regimen and that achievement of complete resolution predicts better long-term outcomes.

  13. [Para-neoplastic autoimmune multi-organ syndrome associated with follicular lymphoma: a case report and literature review].

    Science.gov (United States)

    Chen, D; Lin, C Y; Han, X; Chen, B; Lu, Z H; Chang, X Y; Duan, M H

    2016-12-14

    Objective: To broaden our knowledge of para-neoplastic autoimmune multi-organ syndrome (PAMS). Methods: A patient with PAMS associated with follicular lymphoma and bronchiolitis obliterans treated in our hospital was retrospectively analyzed and the clinical features of PAMS were reviewed. Results: A 49-year-old female patient suffered from painful ulcers in the oral cavity and vagina, dry cough and dyspnea. Imaging examinations suggested multiple lymph node enlargements. Inguinal lymph node biopsy revealed follicular lymphoma. Although the oral and vaginal ulcers went into remission with glucocorticoid and thalidomide therapy and follicular lymphoma gained partial remission with six cycles of R-CHOP (rituximab, cyclophosphamide, doxorubicin, vincristine, prednisolone) chemotherapy, respiratory failure still progressed. Conclusion: PAMS should be considered in patients with unexplained oral mucosa ulcers and dyspnea, which didn't match with the chest image manifestations. Extensive work-up should be performed to find out the potential tumor after diagnosis of PAMS. Early diagnosis and complete removal of tumor were essential to PAMS treatment.

  14. Thyroid follicular carcinoma presenting as metastatic skin nodules

    Directory of Open Access Journals (Sweden)

    Asad Jehangir

    2015-02-01

    Full Text Available Background: Follicular thyroid cancer (FTC metastasizes most commonly to the lungs and non-cranial bones. Skull and skin are uncommon sites and usually manifest well after the diagnosis of primary malignancy. Metastasis to skull and skin as the presenting feature of FTC is infrequently reported in the literature. Case presentation: A 65-year-old Caucasian woman with a history of thyroid nodule presented with the complaint of rapidly growing skull nodules which had been present for 3 years but were stable previously. She denied any fevers, chills, history of trauma, or weight loss. She denied any history of smoking or head and neck irradiation. On physical examination, she had two non-tender gray cystic lesions – one on her left temporal region and the other on the right parietal region. Biopsy was consistent with metastatic FTC. Magnetic resonance imaging of the brain demonstrated 7.1×3.8 cm and 3.7×4.5 cm fairly homogeneous, enhancing, relatively well-defined masses centered in the posterior and left anterior lateral calvarium with intracranial and extracranial extensions but without any vasogenic edema or mass effect on the brain. Thyroid ultrasound showed numerous nodules in both lobes. The patient underwent a total thyroidectomy. Histopathological studies of the thyroid gland revealed a well-differentiated FTC in the left lobe. Then she underwent resection of the tumor in multiple stages. She did not have any recurrence of the FTC or metastases during the follow-up period and will be receiving radioactive iodine treatment. Conclusion: Bone and lung are the common sites of metastasis from FTC, but involvement of skull or skin is unusual, particularly as the presenting feature. Metastases from FTC should be in the differential of patients with new osteolytic hypervascular skull lesions or cutaneous lesions in head and neck area.

  15. [Urogenital lymphoma presenting with obstructive anuria].

    Science.gov (United States)

    Rabii, Redouane; Mezzour, Mohamed Hicham; Guessous, Hicham; Essaki, Hicham; Joual, Abdenbi; Rachid, Mohamed; Quessar, Asmaa; Benchekroun, Said; El Mrini, Mohamed

    2004-02-01

    The authors report a case of urogenital lymphoma with multiple sites in a patient presenting with oligo-anuria. Clinical examination revealed a hard hypogastric and prostatic mass and an enlarged left scrotum. Abdominopelvic and scrotal ultrasound demonstrated a prostatic tumour, a hypogastric mass, hepatic nodular lesions and coeliac lymphadenopathy with bilateral ureterohydronephrosis and a heterogeneous intrascrotal mass in contact with the lower pole of the left testis. The laboratory assessment revealed severe renal failure. After a haemodialysis session and ultrasound-guided right percutaneous nephrostomy, pelvic magnetic resonance imaging (MRI) showed a very large pelvic mass between the bladder and the rectum and transrectal biopsy of the mass confirmed the diagnosis of high-grade malignant non-Hodgkin's lymphoma (NHL) with a type B lymphoblastic phenotype. Treatment consisted of chemotherapy according to the LMB 93 protocol. The course was favourable with return of normal renal function and complete remission 1 month after induction. The patient is currently in complete remission with a follow-up of 12 months. In the light of this case and a review of the literature, the authors discuss the diagnostic, therapeutic and prognostic aspects of this rare site of lymphoma.

  16. Peripheral T-cell lymphomas of follicular helper T-cell type frequently display an aberrant CD3(-/dim)CD4(+) population by flow cytometry: an important clue to the diagnosis of a Hodgkin lymphoma mimic.

    Science.gov (United States)

    Alikhan, Mir; Song, Joo Y; Sohani, Aliyah R; Moroch, Julien; Plonquet, Anne; Duffield, Amy S; Borowitz, Michael J; Jiang, Liuyan; Bueso-Ramos, Carlos; Inamdar, Kedar; Menon, Madhu P; Gurbuxani, Sandeep; Chan, Ernest; Smith, Sonali M; Nicolae, Alina; Jaffe, Elaine S; Gaulard, Philippe; Venkataraman, Girish

    2016-10-01

    Nodal follicular helper T-cell-derived lymphoproliferations (specifically the less common peripheral T-cell lymphomas of follicular type) exhibit a spectrum of histologic features that may mimic reactive hyperplasia or Hodgkin lymphoma. Even though angioimmunoblastic T-cell lymphoma and peripheral T-cell lymphoma of follicular type share a common biologic origin from follicular helper T-cells and their morphology has been well characterized, flow cytometry of peripheral T-cell lymphomas of follicular type has not been widely discussed as a tool for identifying this reactive hyperplasia/Hodgkin lymphoma mimic. We identified 10 peripheral T-cell lymphomas of follicular type with available flow cytometry data from five different institutions, including two cases with peripheral blood evaluation. For comparison, we examined flow cytometry data for 8 classical Hodgkin lymphomas (including 1 lymphocyte-rich classical Hodgkin lymphoma), 15 nodular lymphocyte predominant Hodgkin lymphomas, 15 angioimmunoblastic T-cell lymphomas, and 26 reactive nodes. Lymph node histology and flow cytometry data were reviewed, specifically for the presence of a CD3(-/dim)CD4(+) aberrant T-cell population (described in angioimmunoblastic T-cell lymphomas), besides other T-cell aberrancies. Nine of 10 (90%) peripheral T-cell lymphomas of follicular type showed a CD3(-/dim)CD4(+) T-cell population constituting 29.3% (range 7.9-62%) of all lymphocytes. Five of 10 (50%) had nodular lymphocyte predominant Hodgkin lymphoma or lymphocyte-rich classical Hodgkin lymphoma-like morphology with scattered Hodgkin-like cells that expressed CD20, CD30, CD15, and MUM1. Three cases had a nodular growth pattern and three others exhibited a perifollicular growth pattern without Hodgkin-like cells. Epstein-Barr virus was positive in 1 of 10 cases (10%). PCR analysis showed clonal T-cell receptor gamma gene rearrangement in all 10 peripheral T-cell lymphomas of follicular type. By flow cytometry, 11 of 15 (73

  17. Radiotherapy as an effective treatment modality for follicular lymphoma: a single institution experience

    Energy Technology Data Exchange (ETDEWEB)

    Choi, Seo Hee; Cho, Jae Ho; Kim, Jin Seok; Cheong, June Won; Suh, Chang Ok [Dept. of Radiation Oncology, Yonsei University College of Medicine, Seoul (Korea, Republic of)

    2015-12-15

    Follicular lymphoma (FL) is an indolent non-Hodgkin's lymphoma that is highly sensitive to radiotherapy (RT). However, the effectiveness of RT has not been well established. We reviewed our experiences to assess the role of RT for FL and analyze treatment results. Retrospective analysis was done on 29 patients who received first RT between January 2003 and August 2013. Of 23 early stage (stage I, II) patients, 16 received RT alone, four received chemotherapy followed by RT, two received RT postoperatively, and one received salvage RT for relapse after resection. Six advanced-stage (stage III, IV) patients received RT after chemotherapy: two received consolidation RT, three received salvage RT for residual lesions, and one received RT for progressive sites. Median RT dose was 30.6 Gy (range, 21.6 to 48.6 Gy). Median follow-up duration was 62 months (range, 6 to 141 months). All patients showed complete response in the radiation field. Eight outfield relapses were reported. Seven patients received salvage treatment (three chemotherapy, four RT). Four patients showed excellent responses, especially to RT. Estimated 5-year and 10-year relapse-free survivals were 72% and 60%. In the RT-alone group, 5-year relapse-free survival was 74.5%. All advanced-stage patients were disease-free with 100% 5-year overall survival. Disease-specific death was noted in only one patient; four others died of other unrelated causes. No significant toxicity was reported. RT resulted in excellent treatment outcomes for all FL stages when used as a primary treatment modality for early stage or salvage-treatment modality for advanced-stage disease.

  18. Hematopoietic stem cell transplantation for follicular lymphoma: optimal timing and indication.

    Science.gov (United States)

    Kim, Sung Won

    2014-01-01

    The definitive management of advanced follicular lymphoma (FL) remains controversial due to various treatment options, including watchful waiting, single-agent or combination chemotherapy, monoclonal antibody, and radioimmunotherapy. These options can provide prolonged progression-free survival. However, they cannot cure advanced FL. Allogeneic hematopoietic stem cell transplantation (allo-SCT) remains the sole curative therapy for FL. Allo-SCT has had a major impact with the use of reduced-intensity conditioning regimens because of its lower associated nonrelapse mortality compared with myeloablative regimens. Autologous SCT (auto-SCT) shows high response rates and extends progression-free survival in patients with chemosensitive relapse. In the rituximab era, however, associated comorbidities, risk of secondary cancers, and presence of refractory disease have become problematic in the auto-SCT population. On the basis of results from large-scale randomized trials, upfront auto-SCT is not recommended. Novel conditioning regimens including radioimmunotherapy followed by either auto-SCT or allo-SCT are likely to show efficacy even in chemorefractory disease. Consequently, the optimal timing for SCT remains a matter of opinion, except for patients in first remission. However, the outcomes of allo-SCT and auto-SCT keep on improving. Physicians should note that there is no therapy with a track record equivalent to that of SCT for relapsed or refractory FL.

  19. Inter-reader variability in follicular lymphoma grading: Conventional and digital reading.

    Science.gov (United States)

    Lozanski, Gerard; Pennell, Michael; Shana'ah, Arwa; Zhao, Weiqiang; Gewirtz, Amy; Racke, Frederick; Hsi, Eric; Simpson, Sabrina; Mosse, Claudio; Alam, Shadia; Swierczynski, Sharon; Hasserjian, Robert P; Gurcan, Metin N

    2013-01-01

    Pathologists grade follicular lymphoma (FL) cases by selecting 10, random high power fields (HPFs), counting the number of centroblasts (CBs) in these HPFs under the microscope and then calculating the average CB count for the whole slide. Previous studies have demonstrated that there is high inter-reader variability among pathologists using this methodology in grading. The objective of this study was to explore if newly available digital reading technologies can reduce inter-reader variability. IN THIS STUDY, WE CONSIDERED THREE DIFFERENT READING CONDITIONS (RCS) IN GRADING FL: (1) Conventional (glass-slide based) to establish the baseline, (2) digital whole slide viewing, (3) digital whole slide viewing with selected HPFs. Six board-certified pathologists from five different institutions read 17 FL slides in these three different RCs. Although there was relative poor consensus in conventional reading, with lack of consensus in 41.2% of cases, which was similar to previously reported studies; we found that digital reading with pre-selected fields improved the inter-reader agreement, with only 5.9% lacking consensus among pathologists. Digital whole slide RC resulted in the worst concordance among pathologists while digital whole slide reading selected HPFs improved the concordance. Further studies are underway to determine if this performance can be sustained with a larger dataset and our automated HPF and CB detection algorithms can be employed to further improve the concordance.

  20. Inter-reader variability in follicular lymphoma grading: Conventional and digital reading

    Directory of Open Access Journals (Sweden)

    Gerard Lozanski

    2013-01-01

    Full Text Available Context: Pathologists grade follicular lymphoma (FL cases by selecting 10, random high power fields (HPFs, counting the number of centroblasts (CBs in these HPFs under the microscope and then calculating the average CB count for the whole slide. Previous studies have demonstrated that there is high inter-reader variability among pathologists using this methodology in grading. Aims: The objective of this study was to explore if newly available digital reading technologies can reduce inter-reader variability. Settings and Design: In this study, we considered three different reading conditions (RCs in grading FL: (1 Conventional (glass-slide based to establish the baseline, (2 digital whole slide viewing, (3 digital whole slide viewing with selected HPFs. Six board-certified pathologists from five different institutions read 17 FL slides in these three different RCs. Results: Although there was relative poor consensus in conventional reading, with lack of consensus in 41.2% of cases, which was similar to previously reported studies; we found that digital reading with pre-selected fields improved the inter-reader agreement, with only 5.9% lacking consensus among pathologists. Conclusions: Digital whole slide RC resulted in the worst concordance among pathologists while digital whole slide reading selected HPFs improved the concordance. Further studies are underway to determine if this performance can be sustained with a larger dataset and our automated HPF and CB detection algorithms can be employed to further improve the concordance.

  1. A patient presenting with spinal cord compression who had two distinct follicular cell type thyroid carcinomas.

    Science.gov (United States)

    Koca, E; Sokmensuer, C; Yildiz, B O; Engin, H; Bozkurt, M F; Aras, T; Barista, I; Gurlek, A

    2004-06-01

    A 61-yr-old woman presented with complaints of weakness and pain in her legs. A magnetic resonance imaging showed a 3 x 5.6 x 7.8 cm mass lesion destructing the T1 and T2 vertebral bodies and compressing the spinal cord. The mass was excised surgically. It was follicular carcinoma metastasis of the cervicodorsal region. Then, she underwent a total thyroidectomy. Pathological examination showed two different types of carcinomas in two different focuses; follicular carcinoma in the left lobe and follicular variant papillary carcinoma in the isthmic lobe. After the operation she was given 100 mCi 131I. This is the first report of a patient who had both metastatic follicular carcinoma and follicular variant papillary carcinoma together.

  2. Evaluation of follicular T-helper cells in primary cutaneous CD4+ small/medium pleomorphic T-cell lymphoma and dermatitis.

    Science.gov (United States)

    Ally, Mina S; Prasad Hunasehally, Ranganna Y; Rodriguez-Justo, Manuel; Martin, Blanca; Verdolini, Roberto; Attard, Natalie; Child, Fiona; Attygalle, Ayoma; Whittaker, Sean; Morris, Stephen; Robson, Alistair

    2013-12-01

    CD4+ small/medium-sized pleomorphic T-cell lymphoma (SMPTCL) is a controversial primary cutaneous lymphoma, in which the candidate neoplastic cells express a follicular T-helper phenotype. We describe 16 cases of SMPTCL and compare expression of PD-1, CXCL-13 and ICOS in these tumors with 40 dermatitis cases. Histopathologic examination and immunocytochemistry were performed for 16 tumors and 40 assorted dermatitis cases. All but one patient presented with solitary lesions. Each biopsy revealed a dense nodular non-epitheliotropic infiltrate of atypical T-cells. Neoplastic cells were CD3+/CD4+/CD8(-)/CD30(-). Cutaneous recurrence occurred in one patient over a median follow up of 8 months (range 5-36). All tumors widely expressed PD-1 and ICOS to a lesser extent. CXCL-13 stained much fewer cells. Of the dermatitis cases, PD-1 (most numerous) and ICOS labeled lymphoid cells in all cases, albeit fewer than in the tumors, and CXCL-13 was negative in 32. A rosette pattern of PD-1 expression was identified in all the SMPTCL cases but not in dermatitis. There remains uncertainty about the appropriate nosological status of SMPTCL, which some authors consider to be a pseudolymphoma. However, this study suggests a significant difference in the prevalence and pattern of follicular T-helper cell markers between this tumor and lymphoid proliferations known to be reactive. © 2013 John Wiley & Sons A/S. Published by John Wiley & Sons Ltd.

  3. Idelalisib therapy of indolent B-cell malignancies: chronic lymphocytic leukemia and small lymphocytic or follicular lymphomas

    Directory of Open Access Journals (Sweden)

    Madanat YF

    2016-03-01

    Full Text Available Yazan F Madanat,1 Mitchell R Smith,2 Alexandru Almasan,3 Brian T Hill2 1Department of Internal Medicine, 2Department of Hematology and Medical Oncology, Taussig Cancer Institute, 3Department of Cancer Biology, Lerner Research Institute, Cleveland Clinic, Cleveland, OH, USA Abstract: Chronic lymphocytic leukemia, small lymphocytic lymphoma, and follicular lymphoma are indolent B-cell lymphoproliferative disorders that mainly affect an older population. Although the majority of patients in need of treatment derive significant benefit from conventional chemotherapeutic agents as well as monoclonal antibodies, less toxic and more effective treatments are needed. Novel agents that inhibit the B-cell receptor signaling pathway have shown promising outcomes in these disorders. Idelalisib is a potent selective oral inhibitor of phosphatidylinositol 3-kinase delta and has shown significant clinical activity in B-cell malignancies. In this review, we summarize the clinical trial data using idelalisib as monotherapy or in combination with rituximab for the treatment of relapsed/refractory disease. The adverse effect profile includes autoimmune disorders such as transaminitis, colitis, and pneumonitis. Given the efficacy and manageable toxicity profile of idelalisib, it is being increasingly incorporated into the management of indolent B-cell malignancies. Keywords: idelalisib, PI3Kδ inhibitors, chronic lymphocytic leukemia, follicular lymphoma

  4. A meta-analysis of genome-wide association studies of follicular lymphoma

    Directory of Open Access Journals (Sweden)

    Skibola Christine F

    2012-10-01

    Full Text Available Abstract Background B-cell non-Hodgkin lymphoma represents a diverse group of hematological malignancies, of which follicular lymphoma (FL is one of the most common subtypes. Family and epidemiological studies suggest an important genetic role in the etiology of FL. In recent genome-wide association studies (GWAS of FL, several genetic susceptibility loci have been identified on chromosome 6p21.33 (rs6457327 and 6p21.32 (rs10484561, rs2647012 in the human leukocyte antigen class I and class II regions. To identify new genetic variants and further elucidate the genetic basis of FL, a meta-analysis was performed of the top 1000 SNPs associated with FL risk from two GWAS in the US, Denmark and Sweden (592 cases, 1541 controls, with independent validation in 107 cases and 681 controls. Results rs9275517 and rs3117222 in the HLA class II region were validated and inversely associated with FL risk (rs9275517: OR = 0.63, 95% CI = 0.55-0.73, p = 4.03 × 10-11; rs3117222: OR = 0.66, 95% CI = 0.57-0.77, p = 1.45 × 10-7. rs9275517, which is in high linkage disequilibrium with rs2647012 (r2 = 0.9, was no longer associated with FL after conditioning on rs2647012. The rs3117222 association was independent of established FL SNPs, but not of the HLA-DPB1*0301 allele. Using publicly available gene expression profiles with matching genotype information, we found that rs3117222 also was significantly correlated with increased HLA-DPB1 expression. Conclusions By performing a meta-analysis of two GWAS of FL, we further validated the relevance of HLA-DPB1*0301 as a protective allele in the pathogenesis of FL. Moreover, the protective rs3117222 A allele correlated with increased levels of HLA-DPB1, suggesting a possible disease mechanism involving HLA-DPB1 expression regulation. Our results add further support to the major role of HLA genetic variation in the pathogenesis of FL.

  5. Morphologic studies of lymphocyte nuclei in follicular and diffuse mixed small- and large-cell (lymphocytic-histiocytic) lymphoma.

    Science.gov (United States)

    Dardick, I; Caldwell, D R; Moher, D; Jabi, M

    1988-08-01

    Twelve examples of mixed small- and large-cell lymphoma (eight follicular, one follicular and diffuse, and three diffuse) were investigated morphometrically using plastic-embedded tissue in order to study nuclear characteristics of lymphocyte populations in this form of non-Hodgkin's lymphoma (NHL) and to test morphologic bases for current NHL classification systems. This study illustrates that there are many inaccuracies, illusions, and misconceptions in the morphologic criteria currently used to classify mixed small- and large-cell lymphoma. A principal finding was that lymphocyte nuclear profiles in mixed-cell lymphomas tend to be smaller in size (P less than .005) and more irregular in shape (P = .0001) than the morphologically similar counterparts in germinal centers of lymph nodes with reactive hyperplasia. Intercase comparison of mixed small- and large-cell lymphomas revealed a considerable range of mean nuclear area values, some of which were within the size range of normal, small lymphocytes. At the magnifications used for morphometric assessment, a high proportion of lymphocyte nuclear profiles had shallow invaginations, but only a limited number of profiles (4% to 14%) had deep (cleaved) indentations. Contrary to current definitions for this subtype of NHL, lymphocytes with "small" nuclei had the same proportion of the nuclear diameter occupied by nuclear invaginations as lymphocytes with "large" nuclei and, in fact, mean nuclear invagination depth was shallower in "small" nuclei than in "large" nuclei. Furthermore, regardless of whether it is nuclear area or shape that is evaluated, lymphocytes in mixed-cell lymphoma do not separate into two populations of small-cleaved and large noncleaved cells. Morphometry reveals that only four of the 12 examples of mixed small- and large-cell lymphoma had a proportion of the lymphocytes in the size range of fully transformed germinal center lymphocytes that exceeded 25%, and none of the cases approached 50% even

  6. Safety and efficacy of Obinutuzumab with CHOP or bendamustine in previously untreated follicular lymphoma.

    Science.gov (United States)

    Grigg, Andrew; Dyer, Martin J S; González Díaz, Marcos; Dreyling, Martin; Rule, Simon; Lei, Guiyuan; Knapp, Andrea; Wassner-Fritsch, Elisabeth; Marlton, Paula

    2016-12-23

    The GAUDI study assessed safety and preliminary efficacy of induction therapy with obinutuzumab plus chemotherapy, followed by maintenance with obinutuzumab alone, in previously untreated patients with follicular lymphoma. Assignment to chemotherapy was decided on a per center basis before patient enrollment. Patients (n=81) received 4-6 cycles of obinutuzumab plus bendamustine every 4 weeks or 6-8 cycles of obinutuzumab plus CHOP every 3 weeks. Patients with an end-of-treatment response were eligible for obinutuzumab maintenance therapy every 3 months for 2 years or until disease progression. Induction treatment was completed by 90% of patients in the obinutuzumab plus bendamustine group and 95% in the obinutuzumab plus CHOP group, while maintenance was completed by 81% and 72% of patients, respectively. All patients experienced at least one adverse event during induction, most commonly infusion-related reactions (58%), the majority of which were grade 1/2. The most common hematologic adverse event was grade 3/4 neutropenia (36% during induction and 7% during maintenance). One treatment-related death occurred during the maintenance phase. At the end of induction, 94% of patients had achieved an overall response, with complete response based on CT in 36%. The progression-free survival rate at 36 months was 90% in the obinutuzumab plus bendamustine group and 84% in the obinutuzumab plus CHOP group. These results demonstrate that induction therapy with obinutuzumab plus bendamustine or obinutuzumab plus CHOP, followed by obinutuzumab maintenance, is associated with tolerable safety and promising efficacy. This study is registered at ClinicalTrials.gov as NCT00825149.

  7. Advanced-stage III/IV follicular lymphoma. Treatment strategies for individual patients

    Energy Technology Data Exchange (ETDEWEB)

    Heinzelmann, Frank; Bamberg, Michael; Weinmann, Martin [Dept. of Radiation Oncology, Univ. of Tuebingen (Germany); Ottinger, Hellmut [Dept. of Bone Marrow Transplantation, Univ. of Essen (Germany); Engelhard, Marianne [Dept. of Radiation Oncology, Univ. of Essen (Germany); Soekler, Martin [Dept. of Internal Medicine II, Univ. of Tuebingen (Germany)

    2010-05-15

    Background: in patients with advanced-stage III/IV follicular lymphoma (FL), there are many treatment options available. The current challenge is to choose the optimal strategy for the individual patient. Methods: the literature was reviewed with respect to treatment strategies in patients with advanced FL by screening the PubMed databank. Results: in advanced-stage III/IV FL, median survival may approach 8-10 years. Treatment strategies include a watch-and-wait strategy, chemoimmunotherapy, monotherapy with rituximab, and - as an experimental approach so far - radioimmunotherapy. The use of autologous hematopoietic stem cell transplantation (HSCT) for patients in first remission or chemosensitive relapse prolongs progression-free survival while the effect on overall survival remains unclear compared to standard chemotherapy. However, long-term results are flawed by high relapse rates and risk of secondary malignancies. In patients with relapsed/chemoresistant disease, allogeneic HSCT constitutes the only curative approach but is associated with high treatment-related mortality. In the palliative setting, low-dose involved-field irradiation constitutes an effective treatment option in order to control local symptoms with potential long-lasting response. Conclusion: in case of advanced-disease FL, asymptomatic patients can be managed expectantly. In symptomatic patients, chemoimmunotherapy is regarded as standard therapy. In symptomatic elderly patients with relevant comorbidities, rituximab {+-} single-agent chemotherapy, or low-dose involved-field radiotherapy might be appropriate. For younger patients with chemoresistant/relapsed disease, allogeneic HSCT might be considered, since advances in supportive care and better patient selection have resulted in improved outcomes. (orig.)

  8. Somatic mutation of EZH2 (Y641) in follicular and diffuse large B-cell lymphomas of germinal center origin | Office of Cancer Genomics

    Science.gov (United States)

    Morin et al. describe recurrent somatic mutations in EZH2, a polycomb group oncogene. The mutation, found in the SET domain of this gene encoding a histone methyltransferase, is found only in a subset of lymphoma samples. Specifically, EZH2 mutations are found in about 12% of follicular lymphomas (FL) and almost 23% of diffuse large B-cell lymphomas (DLBCL) of germinal center origin. This paper goes on to demonstrate that altered EZH2 proteins, corresponding to the most frequent mutations found in human lymphomas, have reduced activity using in vitro histone methylation assays.

  9. GWAS of follicular lymphoma reveals allelic heterogeneity at 6p21.32 and suggests shared genetic susceptibility with diffuse large B-cell lymphoma

    DEFF Research Database (Denmark)

    Smedby, Karin E; Foo, Jia Nee; Skibola, Christine F;

    2011-01-01

    Non-Hodgkin lymphoma (NHL) represents a diverse group of hematological malignancies, of which follicular lymphoma (FL) is a prevalent subtype. A previous genome-wide association study has established a marker, rs10484561 in the human leukocyte antigen (HLA) class II region on 6p21.32 associated...... with increased FL risk. Here, in a three-stage genome-wide association study, starting with a genome-wide scan of 379 FL cases and 791 controls followed by validation in 1,049 cases and 5,790 controls, we identified a second independent FL-associated locus on 6p21.32, rs2647012 (OR(combined)  = 0.64, P...

  10. PET/CT assessment in follicular lymphoma using standardized criteria: central review in the PRIMA study

    Energy Technology Data Exchange (ETDEWEB)

    Tychyj-Pinel, Christelle [Service de Medecine Nucleaire, Hospices Civils de Lyon, Centre Hospitalier Lyon Sud, Pierre-Benite (France); Ricard, Fabien [Service de Medecine Nucleaire, Hospices Civils de Lyon, Centre Hospitalier Lyon Sud, Pierre-Benite (France); Universite de Lyon, Faculte de Medecine, UCB Lyon 1, Lyon (France); Fulham, Michael [Royal Prince Alfred Hospital, Department of PET and Nuclear Medicine, Sydney (Australia); University of Sydney, Sydney Medical School, Sydney (Australia); Fournier, Marion [Centre Hospitalier Lyon Sud, The Lymphoma Academic Research Organisation (LYSARC), Pierre-Benite (France); Meignan, Michel [CHU Henri Mondor, Medicine Nucleaire, Creteil (France); Lamy, Thierry [Service d' Hematologie, CHU, Rennes (France); Vera, Pierre [Centre Henri Becquerel, Service de Medecine Nucleaire, Rouen (France); Rouen University, QuantIF (Litis EA4108), Rouen (France); Salles, Gilles [Universite de Lyon, Faculte de Medecine, UCB Lyon 1, Lyon (France); Service d' Hematologie, Hospices Civils de Lyon, Centre Hospitalier Lyon Sud, Pierre-Benite (France); Trotman, Judith [University of Sydney, Sydney Medical School, Sydney (Australia); Concord Hospital, Department of Haematology, Concord, NSW (Australia)

    2014-03-15

    We aimed to compare the standardized central review of {sup 18}F-fluorodeoxyglucose (FDG) positron emission tomography (PET)/CT scans performed after induction therapy for follicular lymphoma (FL) in the PRIMA study (Salles et al., Lancet 377:42-51, 2011; Trotman et al., J Clin Oncol 29:3194-3200, 2011) to scan review at local centres. PET/CT scans were independently evaluated by two nuclear medicine physicians using the 2007 International Harmonization Project (IHP) criteria (Cheson et al., J Clin Oncol 25:579-586, 2007; Juweid et al., J Clin Oncol 25:571-578, 2007; Shankar et al., J Nucl Med 47:1059-1066, 2006) and Deauville 5-point scale (5PS) criteria (Meignan et al., Leuk Lymphoma 50:1257-1260, 2009; Meignan et al., Leuk Lymphoma 51:2171-2180, 2010; Barrington et al., Eur J Nucl Med Mol Imaging 37:1824-1833, 2010). PET/CT status was compared with prospectively recorded patient outcomes. Central evaluation was performed on 119 scans. At diagnosis, 58 of 59 were recorded as positive, with a mean maximum standardized uptake value (SUV{sub max}) of 11.7 (range 4.6-35.6). There was no significant association between baseline SUV{sub max} and progression-free survival (PFS). Sixty post-induction scans were interpreted using both the IHP criteria and 5PS. Post-induction PET-positive status failed to predict progression when applying the IHP criteria [p = 0.14; hazard ratio (HR) 1.9; 95 % confidence interval (CI) 0.8-4.6] or 5PS with a cut-off ≥3 (p = 0.12; HR 2.0; 95 % CI 0.8-4.7). However, when applying the 5PS with a cut-off ≥4, there was a significantly inferior 42-month PFS in PET-positive patients of 25.0 % (95 % CI 3.7-55.8 %) versus 61.4 % (95 % CI 45.4-74.1 %) in PET-negative patients (p = 0.01; HR 3.1; 95 % CI 1.2-7.8). The positive predictive value (PPV) of post-induction PET with this liver cut-off was 75 %. The 42-month PFS for patients remaining PET-positive by local assessment was 31.1 % (95 % CI 10.2-55.0 %) vs 64.6 % (95 % CI 47.0-77.6 %) for PET

  11. Pediatric Burkitt lymphoma presenting as acute pancreatitis: MRI characteristics

    Energy Technology Data Exchange (ETDEWEB)

    Amodio, John; Brodsky, Jennie E. [SUNY Downstate Medical Center, Department of Radiology, Brooklyn, NY (United States)

    2010-05-15

    Acute pancreatitis is a rare initial presentation of non-Hodgkin lymphoma with few reported cases described in older adults and even fewer in children. MRI features of Burkitt lymphoma of the pancreas are sparse in the radiologic literature. We present a 6-year-old boy who presented with pancreatitis and obstructive jaundice, which was the result of Burkitt lymphoma of the pancreas. The imaging findings of pancreatic involvement of Burkitt lymphoma on MRI are discussed and the contributory role of the radiologist in guiding the appropriate clinical work-up of this disease is highlighted. (orig.)

  12. CD10 down expression in follicular lymphoma correlates with gastrointestinal lesion involving the stomach and large intestine.

    Science.gov (United States)

    Ohnishi, Nobuhiko; Takata, Katsuyoshi; Miyata-Takata, Tomoko; Sato, Yasuharu; Tari, Akira; Gion, Yuka; Noujima-Harada, Mai; Taniguchi, Kohei; Tabata, Tetsuya; Nagakita, Keina; Omote, Shizuma; Takahata, Hiroyuki; Iwamuro, Masaya; Okada, Hiroyuki; Maeda, Yoshinobu; Yanai, Hiroyuki; Yoshino, Tadashi

    2016-11-01

    Follicular lymphoma (FL) shows co-expression of B-cell lymphoma 2 (BCL2) and CD10, whereas downexpression of CD10 is occasionally experienced in gastrointestinal (GI) FL with unknown significance. Gastrointestinal FL is a rare variant of FL, and its similarity with mucosa-associated lymphoid tissue lymphoma was reported. We investigated the clinicopathological and genetic features of CD10 downexpressed (CD10(down) ) GI-FL. The diagnosis of CD10(down) FL was carried out with a combination of pathological and molecular analyses. The incidence of CD10(down) GI-FL was shown in 35/172 (20.3%) cases, which was more frequent than nodal FL (3.5%, P stomach or large intestine (P = 0.015), and additionally showed the downexpression of BCL6 (P < 0.001). The follicular dendritic cell meshwork often showed a duodenal pattern in the CD10(down) group (P = 0.12). Furthermore, a lymphoepithelial lesion was observed in 5/12 (40%) gastric FL cases, which indicated caution in the differentiation of mucosa-associated lymphoid tissue lymphoma. Molecular analyses were undertaken in seven cases of CD10(down) GI-FL, and an identical clone was found between CD10(down) follicles and CD10(+) BCL2(+) neoplastic follicles. In the diagnosis of cases with CD10(down) BCL2(+) follicles, careful examination with molecular studies should be carried out. © 2016 The Authors. Cancer Science published by John Wiley & Sons Australia, Ltd on behalf of Japanese Cancer Association.

  13. The somatic hypermutation activity of a follicular lymphoma links to large insertions and deletions of immunoglobulin genes.

    Science.gov (United States)

    Wu, H Y; Kaartinen, M

    1995-07-01

    A biopsy specimen from a patient with follicular lymphoma was divided into two fragments. DNA was extracted from one fragment and a 1.2 kb region of the functional heavy chain (IgH) gene was amplified, cloned and sequenced (eight clones). From the other fragment a cell line (HF-1) was started. The IgH gene region was amplified from the cell line, and sequenced without cloning. The nine sequences obtained could be arranged into a genealogical tree where the individual sequences differed from the deduced ancestor by 16-29 single nucleotide changes, some also by an insertion and/or a deletion. It is apparent that the sequence alterations were caused by somatic mutations during the growth of the lymphoma. The comparison of the sequences with two published (allelic) germline sequences of the human JH region showed approximately 20% non-homology. The differences included five additional multinucleotide insertion/deletion changes, the longest of them a 101-nucleotide insertion. Two long insertions were homologous to the adjacent germline sequences. We propose that most of the changes observed, including long deletions and insertions, represent or are linked to somatic hypermutation events of the Ig gene type. Although in a few cases large deletions and insertions (> 2 bp) have been found in mutated immunoglobulin genes, our results, for the first time, firmly link these deletions/insertions to somatic hypermutations; their frequency was found to be 2.2% of the observed mutational events in the non-translated gene regions. HF-1 is the first follicular lymphoma line successfully established from a lymphoma known to have hypermutated its Ig genes during the malignant growth. It is a candidate cell line to be studied for its ability to generate mutations of B cell type in cell cultures.

  14. An intra-thoracic follicular carcinoma of thyroid: An uncommon presentation.

    Science.gov (United States)

    Kant, Surya; Srivastava, Anand; Kumar, Rahul; Verma, Ajay Kumar; Mishra, Anand Kumar; Husain, Nuzhat

    2017-01-01

    Follicular carcinoma of thyroid is the second most common type of carcinoma of thyroid, and it may metastasize to bone, lung, brain, and skin. However, the initial presentation of follicular carcinoma of the thyroid as a large intrathoracic mass without any symptoms of thyroid gland enlargement and dysfunction is very rare. We hereby report a case of a 50-year-old male who presented with chief complaints of chest and low back pain. Preliminary evaluation led to the provisional diagnosis of left-sided intrathoracic mass with vertebral metastasis which was suspected to be a case of bronchogenic carcinoma with distant metastasis. Surprisingly, transthoracic biopsy and histopathology revealed metastasis from follicular carcinoma of thyroid. This prompted us for a retrograde evaluation for a primary thyroid malignancy for which an ultrasound and contrast enhanced computed tomography (CECT) of the neck was done which confirmed the presence of a solitary thyroid nodule. Ultrasonography-guided fine-needle aspiration cytology of the nodule revealed follicular carcinoma of thyroid. Histopathological evaluation subsequent to total thyroidectomy revealed follicular carcinoma thyroid, further confirming the diagnosis. The patient was then referred to Department of Nuclear Medicine and Radiotherapy for radionuclide ablation and chemotherapy. We chose to report this case because of its rare presentation as a large intrathoracic mass and the retrograde diagnosis of follicular carcinoma of thyroid. To the best of our knowledge, this is the first report of such a rare case.

  15. A population-based study of prognosis in advanced stage follicular lymphoma managed by watch and wait.

    Science.gov (United States)

    El-Galaly, Tarec Christoffer; Bilgrau, Anders E; de Nully Brown, Peter; Mylam, Karen J; Ahmad, Syed A; Pedersen, Lars M; Gang, Anne O; Bentzen, Hans H; Juul, Maja B; Bergmann, Olav J; Pedersen, Robert S; Nielsen, Berit J; Johnsen, Hans E; Dybkaer, Karen; Bøgsted, Martin; Hutchings, Martin

    2015-05-01

    Watch and wait (WAW) is a common approach for asymptomatic, advanced stage follicular lymphoma (FL), but single-agent rituximab is an alternative for these patients. In this nationwide study we describe the outcome of patients selected for WAW. A cohort of 286 out of 849 (34%) stage III-IVA FL patients seen between 2000 and 2011, were managed expectantly and included. The 5-year progression-free survival (PFS) was 35% [95% confidence interval (CI) 29-42]. The 10-year overall survival (OS) was 65% (95%CI 54-78), and the cumulative risk of dying from lymphoma within 10 years of diagnosis was 13% (95%CI 7-20). Elevated lactate dehydrogenase and > four nodal regions involved were associated with a higher risk of lymphoma treatment and death from lymphoma. The WAW patients and a matched background population had similar OS during the first 50 months after diagnosis (P = 0·7), but WAW patients had increased risk of death after 50 months (P < 0·001). The estimated loss of residual life after 10 years was 6·8 months. The 10-year cumulative risk of histological transformation was 22% (95%CI 15-29) and the 3-year OS after transformation was 71% (95%CI 58-87%). In conclusion, advanced stage FL managed by WAW had a favourable outcome and abandoning this strategy could lead to overtreatment in some patients.

  16. THE ROLE OF AUTOLOGOUS AND ALLOGENEIC STEM CELL TRANSPLANTATION IN FOLLICULAR LYMPHOMA IN THE NEW DRUGS ERA.

    Directory of Open Access Journals (Sweden)

    Francesco Maura

    2016-09-01

    Full Text Available Follicular lymphoma (FL is the second most common histotype of non-Hodgkin’s lymphoma and it is generally characterized by a heterogeneous clinical course. Despite recent therapeutic and diagnostic improvements, a significant fraction of FL patients still relapsed. In younger and/or fit FL relapsed patients bone marrow transplant (BMT has represented the main salvage therapy for many years. Thanks to the ability of high dose chemotherapy to overcome the lymphoma resistance and refractoriness, autologous stem cell transplantation (ASCT is able to achieve a high complete remission rate (CR and favourable outcome in terms of progression free survival (PFS and overall survival (OS. Allogeneic stem cell transplantation (alloSCT combines the high dose chemotherapy effect together with the immune reaction of the donor immune system against lymphoma, the so called ‘graft versus lymphoma’ (GVL effect. Considering the generally higher transplant related mortality (TRM, alloSCT is mostly indicated for FL relapsed after ASCT. During the last years there has been a great spread of novel effective and feasible drugs Although these and future novel drugs will probably change our current approach to FL, the OS post-BMT (ASCT and alloSCT has never been reproduced by any novel combination. In this scenario, it is important to correctly evaluate the disease status, the relapse risk and the comorbidity profile of the relapsed FL patients in order to provide the best salvage therapy and eventually transplant consolidation.

  17. Obinutuzumab, Venetoclax, and Lenalidomide in Treating Patients With Relapsed or Refractory B-cell Non-Hodgkin Lymphoma

    Science.gov (United States)

    2017-03-01

    B-Cell Lymphoma, Unclassifiable, With Features Intermediate Between Diffuse Large B-Cell Lymphoma and Burkitt Lymphoma; Grade 1 Follicular Lymphoma; Grade 2 Follicular Lymphoma; Grade 3a Follicular Lymphoma; Recurrent Burkitt Lymphoma; Recurrent Diffuse Large B-Cell Lymphoma; Recurrent Follicular Lymphoma; Recurrent Marginal Zone Lymphoma; Refractory Burkitt Lymphoma; Refractory Diffuse Large B-Cell Lymphoma; Refractory Follicular Lymphoma; Transformed Recurrent Non-Hodgkin Lymphoma

  18. T-Cell Lymphomas Presenting as Colon Ulcers and Eosinophilia

    Directory of Open Access Journals (Sweden)

    Ping-Hsiu Wu

    2015-07-01

    Full Text Available Primary gastrointestinal T-cell lymphoma is an uncommon entity and primary colon T-cell lymphoma is even rarer. The majority of enteropathy-associated T-cell lymphomas present predominantly as ulcers or strictures in the endoscopic examinations, while primary B-cell lymphomas commonly present as exophytic lesions. Ulcerative colon T-cell lymphoma may mimic Crohn's disease (CD, which is a chronic inflammatory disease of the intestines with ulcer and fistula formations difficult for clinicians to diagnose based on endoscopic observations alone. Like CD, T-cell lymphoma may be characterized by the presence of multiple skipped ulcers distributed from the terminal ileum to the descending colon. Furthermore, it is difficult to diagnose this unusual lymphoma by a single endoscopic biopsy. Typically, the histological composition of T-cell lymphoma is made of medium to large atypical cells located in the base of the ulcer with extension to the muscle layer and the adjacent mucosa. However, it is common that biopsy specimens show only mixed inflammatory changes where the lymphoma cells are hard to be identified. The differential diagnosis of malignant lymphoma must be considered when clinically diagnosed CD is refractory to the medical treatment or when its clinical behavior becomes aggressive. The current study presents a rare case of primary colon T-cell lymphoma in a 56-year-old male with marked recent weight loss, watery diarrhea and bilateral neck lymphadenopathy, who received a laboratory checkup and endoscopic workup for colon biopsy. The initial pathological report was consistent with mucosal inflammation and benign colon ulcers. Interestingly, the blood test showed a prominent eosinophilia. A biopsy of the enlarged neck lymph nodes done approximately 1 month after the colon biopsy unexpectedly showed T-cell lymphoma, which led to a review of the initial colonic biopsy specimens. Additional immunohistochemical stains were used accordingly, which

  19. In situ analysis of the variable heavy chain gene of an IgM/IgG-expressing follicular lymphoma - Evidence for interfollicular trafficking of tumor cells

    NARCIS (Netherlands)

    Aarts, WM; Bende, RJ; Vaandrager, JW; Kluin, PM; Langerak, AW; Pals, ST; van Noesel, CJM

    2002-01-01

    is generally assumed that follicular lymphomas (FL) not only morphologically resemble normal germinal centers but have retained some functional characteristics of their non-neoplastic counterparts as well. Recent IgV gene analyses on a panel of FLs however, strongly suggested that FLs do not retain

  20. Single-institution long-term outcomes for patients receiving nonmyeloablative conditioning hematopoeitic cell transplantation for chronic lymphocytic leukemia and follicular lymphoma

    DEFF Research Database (Denmark)

    Mortensen, Bo K; Petersen, Søren; Kornblit, Brian;

    2012-01-01

    Non-myeloablative conditioning hematopoietic cell transplantation (NMC-HCT) has improved the treatment of chronic lymphocytic leukemia (CLL) and follicular lymphoma (FL). In a cohort of 85 patients (45 with CLL and 40 with FL), we observed 5-yr overall survival (OS) and progression-free survival ...

  1. Small B cell lymphocytic lymphoma presenting as obstructive sleep apnea

    Directory of Open Access Journals (Sweden)

    Chang Weng-Cheng

    2004-07-01

    Full Text Available Abstract Background Most lymphomas that involve the tonsil are large B cell lymphomas. Large B-cell lymphoma is a high grade malignancy which progresses rapidly. Tonsillar lymphoma usually presents as either a unilaterally enlarged palatine tonsil or as an ulcerative and fungating lesion over the tonsillar area. Small lymphocytic lymphomas (SLL of the Waldeyer's ring are uncommon. Case presentation We report a 41-year-old male who presented with a ten-year history of snoring. Physical examination revealed smooth bilateral symmetrically enlarged tonsils without abnormal surface change or cervical lymphadenopathy. Palatal redundancy and a narrowed oropharyngeal airway were also noted. The respiratory disturbance index (RDI was 66 per hour, and severe obstruction sleep apnea (OSA was suspected. No B symptoms, sore throat, odynophagia or dysphagia was found. We performed uvulopalatopharyngoplasty (UPPP and pathological examination revealed incidental small B-cell lymphocytic lymphoma (SLL. Conclusion It is uncommon for lymphoma to initially present as OSA. SLL is an indolent malignancy and is not easy to detect in the early stage. We conclude that SLL may be a contributing factor of OSA in the present case.

  2. GWAS of follicular lymphoma reveals allelic heterogeneity at 6p21.32 and suggests shared genetic susceptibility with diffuse large B-cell lymphoma.

    Science.gov (United States)

    Smedby, Karin E; Foo, Jia Nee; Skibola, Christine F; Darabi, Hatef; Conde, Lucia; Hjalgrim, Henrik; Kumar, Vikrant; Chang, Ellen T; Rothman, Nathaniel; Cerhan, James R; Brooks-Wilson, Angela R; Rehnberg, Emil; Irwan, Ishak D; Ryder, Lars P; Brown, Peter N; Bracci, Paige M; Agana, Luz; Riby, Jacques; Cozen, Wendy; Davis, Scott; Hartge, Patricia; Morton, Lindsay M; Severson, Richard K; Wang, Sophia S; Slager, Susan L; Fredericksen, Zachary S; Novak, Anne J; Kay, Neil E; Habermann, Thomas M; Armstrong, Bruce; Kricker, Anne; Milliken, Sam; Purdue, Mark P; Vajdic, Claire M; Boyle, Peter; Lan, Qing; Zahm, Shelia H; Zhang, Yawei; Zheng, Tongzhang; Leach, Stephen; Spinelli, John J; Smith, Martyn T; Chanock, Stephen J; Padyukov, Leonid; Alfredsson, Lars; Klareskog, Lars; Glimelius, Bengt; Melbye, Mads; Liu, Edison T; Adami, Hans-Olov; Humphreys, Keith; Liu, Jianjun

    2011-04-01

    Non-Hodgkin lymphoma (NHL) represents a diverse group of hematological malignancies, of which follicular lymphoma (FL) is a prevalent subtype. A previous genome-wide association study has established a marker, rs10484561 in the human leukocyte antigen (HLA) class II region on 6p21.32 associated with increased FL risk. Here, in a three-stage genome-wide association study, starting with a genome-wide scan of 379 FL cases and 791 controls followed by validation in 1,049 cases and 5,790 controls, we identified a second independent FL-associated locus on 6p21.32, rs2647012 (OR(combined)  = 0.64, P(combined)  = 2 × 10(-21)) located 962 bp away from rs10484561 (r(2)<0.1 in controls). After mutual adjustment, the associations at the two SNPs remained genome-wide significant (rs2647012:OR(adjusted)  = 0.70, P(adjusted)  =  4 × 10(-12); rs10484561:OR(adjusted)  = 1.64, P(adjusted)  = 5 × 10(-15)). Haplotype and coalescence analyses indicated that rs2647012 arose on an evolutionarily distinct haplotype from that of rs10484561 and tags a novel allele with an opposite (protective) effect on FL risk. Moreover, in a follow-up analysis of the top 6 FL-associated SNPs in 4,449 cases of other NHL subtypes, rs10484561 was associated with risk of diffuse large B-cell lymphoma (OR(combined)  = 1.36, P(combined)  =  1.4 × 10(-7)). Our results reveal the presence of allelic heterogeneity within the HLA class II region influencing FL susceptibility and indicate a possible shared genetic etiology with diffuse large B-cell lymphoma. These findings suggest that the HLA class II region plays a complex yet important role in NHL.

  3. Human Leukocyte Antigen Class I and II Alleles and Overall Survival in Diffuse Large B-Cell Lymphoma and Follicular Lymphoma

    Directory of Open Access Journals (Sweden)

    Yani Lu

    2011-01-01

    Full Text Available Genetic variation in the 6p21 chromosomal region, including human leukocyte antigen (HLA genes and tumor necrosis factor (TNF, has been linked to both etiology and clinical outcomes of lymphomas. We estimated the effects of HLA class I (A, B, and C, class II DRB1 alleles, and the ancestral haplotype (AH 8.1 (HLAA*01-B*08-DRB1*03-TNF-308A on overall survival (OS among patients with diffuse large B-cell lymphoma (DLBCL and follicular lymphoma (FL in a population-based study of non-Hodgkin lymphoma. During a median followup of 89 months, 31% (52 of 166 DLBCL and 28% (46 of 165 FL patients died. Using multivariate Cox regression models, we observed statistically significant associations between genetic variants and survival: HLA-Cw*07:01 was associated with poorer OS among DLBCL patients (Hazard ratio [HR] = 1.76, 95% confidence interval [CI] = 1.01–3.05; HLA-A*01:01 was associated with poorer OS (HR = 2.23, 95% CI = 1.24–4.01, and HLA-DRB1*13 (HR = 0.12, 95% CI = 0.02–0.90 and HLA-B Bw4 (HR = 0.36, 95% CI = 0.20–0.63 with better OS among FL patients. These results support a role for HLA in the prognosis of DLBCL and FL and represent a promising class of prognostic factors that warrants further evaluation.

  4. GWAS of follicular lymphoma reveals allelic heterogeneity at 6p21.32 and suggests shared genetic susceptibility with diffuse large B-cell lymphoma.

    Directory of Open Access Journals (Sweden)

    Karin E Smedby

    2011-04-01

    Full Text Available Non-Hodgkin lymphoma (NHL represents a diverse group of hematological malignancies, of which follicular lymphoma (FL is a prevalent subtype. A previous genome-wide association study has established a marker, rs10484561 in the human leukocyte antigen (HLA class II region on 6p21.32 associated with increased FL risk. Here, in a three-stage genome-wide association study, starting with a genome-wide scan of 379 FL cases and 791 controls followed by validation in 1,049 cases and 5,790 controls, we identified a second independent FL-associated locus on 6p21.32, rs2647012 (OR(combined  = 0.64, P(combined  = 2 × 10(-21 located 962 bp away from rs10484561 (r(2<0.1 in controls. After mutual adjustment, the associations at the two SNPs remained genome-wide significant (rs2647012:OR(adjusted  = 0.70, P(adjusted  =  4 × 10(-12; rs10484561:OR(adjusted  = 1.64, P(adjusted  = 5 × 10(-15. Haplotype and coalescence analyses indicated that rs2647012 arose on an evolutionarily distinct haplotype from that of rs10484561 and tags a novel allele with an opposite (protective effect on FL risk. Moreover, in a follow-up analysis of the top 6 FL-associated SNPs in 4,449 cases of other NHL subtypes, rs10484561 was associated with risk of diffuse large B-cell lymphoma (OR(combined  = 1.36, P(combined  =  1.4 × 10(-7. Our results reveal the presence of allelic heterogeneity within the HLA class II region influencing FL susceptibility and indicate a possible shared genetic etiology with diffuse large B-cell lymphoma. These findings suggest that the HLA class II region plays a complex yet important role in NHL.

  5. Metastatic spinal cord compression as initial presentation of follicular thyroid carcinoma.

    Science.gov (United States)

    Goldberg, H; Stein, M E; Ben-Itzhak, O; Duek, D; Ravkin, A; Gaitini, D

    1998-03-01

    Follicular thyroid carcinoma, initially presenting as spinal cord compression due to metastatic lesions, is a less reported event. We present two cases of well-differentiated thyroid carcinoma that led to spinal cord compression. A thorough search of the literature revealed only five similar cases. We summarize the clinical characteristics of these cases, the therapeutic measures used, their outcome, and the prognosis.

  6. UNCOMMON PRESENTATION OF FOLLICULAR THYROID CARCINOMA: WHEN CHRONIC BACK PAIN SHOULD RAISE A FLAG.

    Science.gov (United States)

    Alvarado, Milliette; Ramirez, Margarita; Lopez, Liurka; Marcos-Martinez, Maria J; Saavedra, Fanor M; Negron-Rivei, Juan C; Agosto, Marielba; Martinez, Meliza; Gonzalez, Rafael; Allende-Vigo, Myriam

    2014-01-01

    Follicular thyroid carcinoma is the second most common type of thyroid cancer, and its incidence has increased dramatically in recent years. Although it typically presents as a thyroid nodule, it can spread to distant sites via hematogenous dissemination. Spinal cord compression complicating thyroid carcinoma is rare with only few cases reported in the literature. This case illustrates a minimally invasive follicular carcinoma that showed such an aggressive behavior, and thus the importance of considering metastatic thyroid carcinoma in the differential diagnosis of chronic back pain, which may possibly progress to spinal cord compression carrying severe morbidity.

  7. An unusual case presentation of follicular odontogenic keratocyst with an impacted mesiodens.

    Science.gov (United States)

    Yadav, Sunil; Verma, Ajay; Sheorain, Anil; Sharma, Arun

    2013-05-01

    Odontogenic keratocyst (OKC) is one of the most aggressive odontogenic cysts known for its high recurrence rate and its tendency to invade adjacent tissues. Radiographically, OKCs can be of different varieties-follicular, envelopmental, replacemental, extraneous, and collateral. Its characteristic clinical and biological behavior, therapeutic approaches, prognosis, and recurrence vary in different studies. Generally, OKCs are found to be associated with impacted lower third molar. Here we present an unusual case of follicular OKC associated with an impacted mesiodens (supernumerary tooth) in a 21-year-old male patient.

  8. A Case of Fulminant Hepatitis due to Echovirus 9 in a Patient on Maintenance Rituximab Therapy for Follicular Lymphoma

    Directory of Open Access Journals (Sweden)

    Ceri Morgan

    2015-01-01

    Full Text Available Rituximab is a CD20 monoclonal antibody commonly used in the treatment of haematological malignancies. It causes lymphopenia with subsequent compromised humoral immunity resulting in an increased risk of infection. A number of infections and viral reactivations have been described as complicating Rituximab therapy. We report an apparently unique case of echovirus 9 (an enterovirus infection causing an acute hepatitis and significant morbidity in an adult patient on maintenance treatment of Rituximab for follicular lymphoma. We also describe potential missed opportunities to employ more robust screening for viral infections which may have prevented delays in the appropriate treatment and thus may have altered the patient’s clinical course. We also make suggestions for lowering the threshold of viral testing in similar patients in the future.

  9. Agatolimod Sodium, Rituximab, and Yttrium Y 90 Ibritumomab Tiuxetan in Treating Patients With Recurrent or Refractory Non-Hodgkin Lymphoma

    Science.gov (United States)

    2016-01-04

    Adult Non-Hodgkin Lymphoma; Extranodal Marginal Zone Lymphoma of Mucosa-Associated Lymphoid Tissue; Nodal Marginal Zone Lymphoma; Recurrent Adult Diffuse Large Cell Lymphoma; Recurrent Grade 1 Follicular Lymphoma; Recurrent Grade 2 Follicular Lymphoma; Recurrent Grade 3 Follicular Lymphoma; Recurrent Mantle Cell Lymphoma; Recurrent Marginal Zone Lymphoma; Recurrent Small Lymphocytic Lymphoma; Splenic Marginal Zone Lymphoma; Waldenstrom Macroglobulinemia

  10. Gene Therapy in Treating Patients With Human Immunodeficiency Virus-Related Lymphoma Receiving Stem Cell Transplant

    Science.gov (United States)

    2016-12-15

    HIV Infection; Mature T-Cell and NK-Cell Non-Hodgkin Lymphoma; Plasmablastic Lymphoma; Recurrent Adult Hodgkin Lymphoma; Recurrent Adult Non-Hodgkin Lymphoma; Recurrent Burkitt Lymphoma; Recurrent Follicular Lymphoma; Stage III Follicular Lymphoma; Stage III Mantle Cell Lymphoma; Stage IV Follicular Lymphoma; Stage IV Mantle Cell Lymphoma

  11. Disseminated lymphoma presenting as acute thigh pain and renal failure.

    LENUS (Irish Health Repository)

    Brown, Catherine

    2009-01-01

    A 66-year-old diabetic man presented with severe right thigh swelling and pain together with acute renal failure. At autopsy, this was found to be due to disseminated high grade B cell lymphoma invading the psoas muscle and multiple organs, including the kidneys. The unique presentation of this case emphasizes the need for increased awareness of the variety of ways in which lymphoma can manifest itself.

  12. Hypercalcaemic multicentric lymphoma in a dog presenting as clitoromegaly

    Directory of Open Access Journals (Sweden)

    Anthony B. Zambelli

    2013-02-01

    Full Text Available Clitoromegaly is a clinical manifestation of various local and systemic conditions in all species. The external genitalia are a very rare site of primary or metastatic lymphoma in canines, with only one previously-reported case in a dog and only sparse reports in the medical literature. Lymphoma is also very rare in dogs less than four years of age. This account reports on a T-cell multicentric lymphoma in a 16-month-old Basset hound presented primarily for clitoromegaly. The patient survived for 68 days with cyclophosphamide-vincristine-prednisolone therapy. The causes of clitoromegaly in all species, including humans, are tabulated with references.

  13. Lymphomas of large cells.

    Science.gov (United States)

    Staples, W G; Gétaz, E P

    1977-09-03

    Historial aspects of the classification of large-cell lymphomas are described. Immunological characterization of the lymphomas has been made possible by identification of T and B lymphocytes according to their cell membrane surface characteristics. The pathogenesis of lymphomas has been clarified by the germinal (follicular) centre cell concepts of Lennert and Lukes and Collins. The various classifications are presented and compared. Whether these subdivisions will have any relevance in the clinical context remains to be seen.

  14. Rituximab purging and/or maintenance in patients undergoing autologous transplantation for relapsed follicular lymphoma

    DEFF Research Database (Denmark)

    Pettengell, Ruth; Schmitz, Norbert; Gisselbrecht, Christian

    2013-01-01

    The objective of this randomized trial was to assess the efficacy and safety of rituximab as in vivo purging before transplantation and as maintenance treatment immediately after high-dose chemotherapy and autologous stem-cell transplantation (HDC-ASCT) in patients with relapsed follicular lympho...

  15. The t(14;18)(q32;q21)/IGH-MALT1 translocation in MALT lymphomas contains templated nucleotide insertions and a major breakpoint region similar to follicular and mantle cell lymphoma.

    Science.gov (United States)

    Murga Penas, Eva Maria; Callet-Bauchu, Evelyne; Ye, Hongtao; Gazzo, Sophie; Berger, Françoise; Schilling, Georgia; Albert-Konetzny, Nadine; Vettorazzi, Eik; Salles, Gilles; Wlodarska, Iwona; Du, Ming-Qing; Bokemeyer, Carsten; Dierlamm, Judith

    2010-03-18

    The t(14;18)(q32;q21) involving the immunoglobulin heavy chain locus (IGH) and the MALT1 gene is a recurrent abnormality in mucosa-associated lymphoid tissue (MALT) lymphomas. However, the nucleotide sequence of only one t(14;18)-positive MALT lymphoma has been reported so far. We here report the molecular characterization of the IGH-MALT1 fusion products in 5 new cases of t(14;18)-positive MALT lymphomas. Similar to the IGH-associated translocations in follicular and mantle cell lymphomas, the IGH-MALT1 junctions in MALT lymphoma showed all features of a recombination signal sequence-guided V(D)J-mediated translocation at the IGH locus. Furthermore, analogous to follicular and mantle cell lymphoma, templated nucleotides (T-nucleotides) were identified at the t(14;18)/IGH-MALT1 breakpoint junctions. On chromosome 18, we identified a novel major breakpoint region in MALT1 upstream of its coding region. Moreover, the presence of duplications of MALT1 nucleotides in one case suggests an underlying staggered DNA-break process not consistent with V(D)J-mediated recombination. The molecular characteristics of the t(14;18)/IGH-MALT1 resemble those found in the t(14;18)/IGH-BCL2 in follicular lymphoma and t(11;14)/CCND1-IGH in mantle cell lymphoma, suggesting that these translocations could be generated by common pathomechanisms involving illegitimate V(D)J-mediated recombination on IGH as well as new synthesis of T-nucleotides and nonhomologous end joining (NHEJ) or alternative NHEJ repair pathways on the IGH-translocation partner.

  16. Entospletinib and Obinutuzumab in Treating Patients With Relapsed Chronic Lymphocytic Leukemia, Small Lymphocytic Lymphoma, or Non-Hodgkin Lymphoma

    Science.gov (United States)

    2017-07-24

    Anemia; B-Cell Prolymphocytic Leukemia; Fatigue; Fever; Grade 1 Follicular Lymphoma; Grade 2 Follicular Lymphoma; Grade 3a Follicular Lymphoma; Hairy Cell Leukemia; Lymphadenopathy; Lymphocytosis; Lymphoplasmacytic Lymphoma; Mantle Cell Lymphoma; Marginal Zone Lymphoma; Night Sweats; Recurrent Chronic Lymphocytic Leukemia; Recurrent Small Lymphocytic Lymphoma; Refractory Chronic Lymphocytic Leukemia; Refractory Small Lymphocytic Lymphoma; Richter Syndrome; Splenomegaly; Thrombocytopenia; Weight Loss

  17. Radiotherapy alone for stage I-III low grade follicular lymphoma: long-term outcome and comparison of extended field and total nodal irradiation

    Directory of Open Access Journals (Sweden)

    Guckenberger Matthias

    2012-06-01

    Full Text Available Abstract Background To analyze long-term results of radiotherapy alone for stage I-III low grade follicular lymphoma and to compare outcome after extended field irradiation (EFI and total nodal irradiation (TNI. Methods and materials Between 1982 and 2007, 107 patients were treated with radiotherapy alone for low grade follicular lymphoma at Ann Arbor stage I (n = 50, II (n = 36 and III (n = 21; 48 and 59 patients were treated with EFI and TNI, respectively. The median total dose in the first treatment series of the diaphragmatic side with larger lymphoma burden was 38 Gy (25 Gy – 50 Gy and after an interval of median 30 days, a total dose of 28 Gy (12.6 Gy – 45 Gy was given in the second treatment series completing TNI. Results After a median follow-up of 14 years for living patients, 10-years and 15-years overall survival (OS were 64% and 50%, respectively. Survival was not significantly different between stages I, II and III. TNI and EFI resulted in 15-years OS of 65% and 34% but patients treated with TNI were younger, had better performance status and higher stage of disease compared to patients treated with EFI. In multivariate analysis, only age at diagnosis (p  Conclusions Radiotherapy alone for stage I and II follicular lymphoma resulted in long-term OS with high rates of disease control; no benefit of TNI over EFI was observed. For stage III follicular lymphoma, TNI achieved promising OS and FFP and should be considered as a potentially curative treatment option.

  18. Anti-CD22 CAR-T Therapy for CD19-refractory or Resistant Lymphoma Patients

    Science.gov (United States)

    2016-08-22

    Recurrent Adult Diffuse Large Cell Lymphoma; Recurrent Follicular Lymphoma; Recurrent Mantle Cell Lymphoma; Stage III/IV Adult Diffuse Large Cell Lymphoma; Stage III/IV Follicular Lymphoma; Stage III/IV Mantle Cell Lymphoma

  19. Correlations between functional imaging markers derived from PET/CT and diffusion-weighted MRI in diffuse large B-cell lymphoma and follicular lymphoma.

    Directory of Open Access Journals (Sweden)

    Xingchen Wu

    Full Text Available OBJECTIVES: To investigate the correlations between functional imaging markers derived from positron emission tomography/computed tomography (PET/CT and diffusion-weighted magnetic resonance imaging (DWI in diffuse large B-cell lymphoma (DLBCL and follicular lymphoma (FL. Further to compare the usefulness of these tumor markers in differentiating diagnosis of the two common types of Non-Hodgkin's lymphoma (NHL. MATERIALS AND METHODS: Thirty-four consecutive pre-therapy adult patients with proven NHL (23 DLBCL and 11 FL underwent PET/CT and MRI examinations and laboratory tests. The maximum standardized uptake value (SUV(max, metabolic tumor volume (MTV, and metabolic tumor burden (MTB were determined from the PET/CT images. DWI was performed in addition to conventional MRI sequences using two b values (0 and 800 s/mm(2. The minimum and mean apparent diffusion coefficient (ADC(min and ADC(mean were measured on the parametric ADC maps. RESULTS: The SUV(max correlated inversely with the ADC(min (r =  -0.35, p<0.05. The ADC(min, ADC(mean, serum thymidine kinase (TK, Beta 2-microglobulin (B2m, lactate dehydrogenase (LD, and C-reactive protein (CRP correlated with both whole-body MTV and whole-body MTB (p<0.05 or 0.01. The SUV(max, TK, LD, and CRP were significantly higher in the DLBCL group than in the FL group. Receiver operating characteristic curve analysis showed that they were reasonable predictors in differentiating DLBCL from FL. CONCLUSIONS: The functional imaging markers determined from PET/CT and DWI are associated, and the SUV(max is superior to the ADC(min in differentiating DLBCL from FL. All the measured serum markers are associated with functional imaging markers. Serum LD, TK, and CRP are useful in differentiating DLBCL from FL.

  20. Relevance of monitoring metabolic reduction in patients with relapsed or refractory follicular and mantle cell lymphoma receiving bendamustine: a multicenter study.

    Science.gov (United States)

    Tateishi, Ukihide; Tatsumi, Mitsuaki; Terauchi, Takashi; Ishizawa, Kenichi; Ogura, Michinori; Tobinai, Kensei

    2011-02-01

    The aim of the present study was to investigate the relevance of monitoring metabolic reduction evaluated by (18) F-fluorodeoxyglucose ((18) F-FDG) PET/CT in relapsed or refractory patients with follicular lymphoma (FL) and mantle cell lymphoma (MCL) who received bendamustine. We conducted a phantom study of 18F-FDG PET/CT to ensure quality control for performing a multicenter clinical study. We analyzed 49 patients with relapsed or refractory FL and MCL who received bendamustine (120 mg/m(2)) on days 1-2 of a 21-day cycle for up to six cycles as a licensing phase II study. 18F-FDG PET/CT scans were acquired before the first and after the last cycle. In a total of 175 target lesions, the maximum perpendicular diameter (Max PD), minimum PD (Min PD), sum of the products of the Max PD (SPD), maximum standardized uptake value (SUVmax), and the percentage reduction rates of Max PD (%Max PD), SPD (%SPD) and SUVmax (%SUVmax) were evaluated for the response to treatment. The therapeutic response was assessed after the last cycle of treatment according to the revised response criteria for malignant lymphoma (revised RC). We evaluated 134 lesions in 39 patients (76%) achieving complete response (CR) and 41 lesions in 10 patients (24%) not achieving CR. The Max PD, Min PD, SPD and SUVmax of the lesions after the last cycle were significantly higher in patients with non-CR than in patients with CR. The %MPD, %SPD and %SUVmax of the lesions were significantly greater in patients with CR than in patients with non-CR (P < 0.0001). Metabolic reduction was observed in all target lesions of relapsed or refractory patients with FL and MCL who achieved CR after bendamustine therapy.

  1. DNA double-strand break rejoining in human follicular lymphoma and glioblastoma tumor cells

    NARCIS (Netherlands)

    Macann, AMJ; Britten, RA; Poppema, S; Pearcey, R; Rosenberg, E; Allalunis-Turner, MJ; Murray, D

    2000-01-01

    Follicle center cell lymphoma is among the most radioresponsive of human cancers. To assess whether this radioresponsiveness might be a result of a compromised ability of the tumor cells to accomplish the biologically-effective repair of DNA double-strand breaks (DSBs), we have measured i) the exten

  2. Interphase FISH detection of BCL2 rearrangement in follicular lymphoma using breakpoint-flanking probes

    NARCIS (Netherlands)

    Vaandrager, J W; Schuuring, E; Raap, T; Philippo, K; Kleiverda, K; Kluin, P

    2000-01-01

    Rearrangement of the BCL2 gene is an important parameter for the differential diagnosis of non-Hodgkin lymphomas. Although a relatively large proportion of breakpoints is clustered, many are missed by standard PCR. A FISH assay is therefore desired. Up to now, a lack of probes flanking the BCL2 gene

  3. Central nervous system lymphoma: magnetic resonance imaging features at presentation.

    Science.gov (United States)

    Schwingel, Ricardo; Reis, Fabiano; Zanardi, Veronica A; Queiroz, Luciano S; França, Marcondes C

    2012-02-01

    This paper aimed at studying presentations of the central nervous system (CNS) lymphoma using structural images obtained by magnetic resonance imaging (MRI). The MRI features at presentation of 15 patients diagnosed with CNS lymphoma in a university hospital, between January 1999 and March 2011, were analyzed by frequency and cross tabulation. All patients had supratentorial lesions; and four had infra- and supratentorial lesions. The signal intensity on T1 and T2 weighted images was predominantly hypo- or isointense. In the T2 weighted images, single lesions were associated with a hypointense signal component. Six patients presented necrosis, all of them showed perilesional abnormal white matter, nine had meningeal involvement, and five had subependymal spread. Subependymal spread and meningeal involvement tended to occur in younger patients. Presentations of lymphoma are very pleomorphic, but some of them should point to this diagnostic possibility.

  4. Memory-enriched CAR-T Cells Immunotherapy for B Cell Lymphoma

    Science.gov (United States)

    2016-04-25

    Recurrent Adult Diffuse Large Cell Lymphoma; Recurrent Follicular Lymphoma; Recurrent Mantle Cell Lymphoma; Stage III Adult Diffuse Large Cell Lymphoma; Stage III Follicular Lymphoma; Stage III Mantle Cell Lymphoma; Stage IV Adult Diffuse Large Cell Lymphoma; Stage IV Follicular Lymphoma; Stage IV Mantle Cell Lymphoma

  5. Burkitts’s lymphoma – an atypical presentation

    Directory of Open Access Journals (Sweden)

    Ziade Farah

    2012-08-01

    Full Text Available Abstract Background In female adolescents and young adults, malignancies of the genital tract are the most frequent type of cancer, closely followed by Hodgkin’s and non-Hodgkin’s lymphomas. Case Presentation We report an unusual case of sporadic Burkitt’s lymphoma (BL presenting with massive bilateral ovarian infiltration, peritoneal carcinomatosis and diffuse nodular lesions of the stomach and the intestine mimicking Krukenberg tumor. Diagnostic biopsies were obtained by endoscopy of the upper gastrointestinal tract. With intensive chemotherapy, complete remission was rapidly achieved, without life-threatening tumor lysis syndrome. Conclusion Besides metastatic gastric adenocarcinoma, BL is an important differential diagnosis in adolescents presenting with Krukenberg tumor.

  6. Basal (18)F-FDG PET/CT in follicular lymphoma: A comparison of metabolic and clinical variables in the prognostic assessment.

    Science.gov (United States)

    Jiménez Londoño, G A; García Vicente, A M; Poblete García, V M; Amo-Salas, M; Calle Primo, C; Ibañez García, Á; Martínez Sanchís, B; López-Fidalgo, J F; Solano Ramos, F; Martínez Hellín, A; Díaz Morfa, M; Soriano Castrejón, Á

    2016-01-01

    To analyze the relationship of clinical variables related to prognosis and tumor burden, with metabolic variables obtained in the staging (18)F-FDG PET/CT, and their value in the prognosis in follicular lymphoma (FL). 82 patients with FL, a (18)F-FDG PET/CT at diagnosis and a follow-up for a minimum of 12 months, were retrospectively enrolled in the present study. Clinical variables (Tumor grade, Follicular Lymphoma International Prognostic Index (FLIPI) and Tumor burden) were evaluated. Metabolic variables such as SUVmax in the highest hypermetabolic lesion, extralymphatic locations, number of involved lymph node locations, bone marrow (BM) involvement, PET stage and diameter of the biggest hypermetabolic lesion, were analyzed in order to establish a PET score and classify the studies in low, intermediate and high metabolic risk. Clinical and metabolic variables (included metabolic risk) were compared. The relation among all variables and disease-free survival (DFS) was studied. The 28% of patients had a high-grade tumor. The 30.5% had FLIPI risk low, 29.3% intermediate y 40.2% high. The 42.7% presented a high tumor burden. The PET/CT was positive in 94% of patients. The tumor grade did not show significant relation with metabolic variable. FLIPI risk and tumor burden showed statistical relations with the SUV max and the PET score (p<0.008 and p=0.003 respectively). With respect to DFS, significant differences were detected for the PET stage and FLIPI risk (p=0.015 and p=0.047 respectively). FLIPI risk was the only significant predictor in Cox regression analysis, with a Hazard Ratio of 5.13 between high risk and low risk. The present research highlights the significant relation between metabolic variables obtained with FDG PET/CT and clinical variables although their goal as an independent factor of prognosis was not demonstrated in the present work. Copyright © 2016 Elsevier España, S.L.U. y SEMNIM. All rights reserved.

  7. Sciatica as a presenting feature of thyroid follicular adenocarcinoma in a 79-year-old woman.

    LENUS (Irish Health Repository)

    Ogbodo, Elisha

    2011-01-01

    The authors describe an unusual case of metastatic thyroid follicular adenocarcinoma presenting with sciatica in a 79-year-old woman. The primary thyroid tumour was undiagnosed until this clinical presentation. The patient gave a short history of back pain and right-sided sciatica, which was progressive and nocturnal in nature. Neuroimaging revealed an enhancing intradural mass lesion, which was completely excised through a right L1-L3 hemilaminectomy. Histopathological examination of the excised tissue revealed a follicular thyroid carcinoma. Subsequent metastatic investigation revealed a heterogeneously attenuating mixed solid cystic mass in a retrosternal thyroid gland, with multiple solid pulmonary nodules suggestive of metastatic disease. She opted for palliative radiotherapy for the primary thyroid cancer and made remarkable postoperative improvement. The authors conclude that surgical treatment of solitary metastatic lesion may produce good symptomatic relief irrespective of patient\\'s age and primary pathology, while emphasising the need for detailed clinical evaluation of patients with \\'red flag\\' symptoms.

  8. Sciatica as a presenting feature of thyroid follicular adenocarcinoma in a 79-year-old woman.

    Science.gov (United States)

    Ogbodo, Elisha; Kaliaperumal, Chandrasekaran; Keohane, Catherine; Bermingham, Niamh; Kaar, George

    2011-12-01

    The authors describe an unusual case of metastatic thyroid follicular adenocarcinoma presenting with sciatica in a 79-year-old woman. The primary thyroid tumour was undiagnosed until this clinical presentation. The patient gave a short history of back pain and right-sided sciatica, which was progressive and nocturnal in nature. Neuroimaging revealed an enhancing intradural mass lesion, which was completely excised through a right L1-L3 hemilaminectomy. Histopathological examination of the excised tissue revealed a follicular thyroid carcinoma. Subsequent metastatic investigation revealed a heterogeneously attenuating mixed solid cystic mass in a retrosternal thyroid gland, with multiple solid pulmonary nodules suggestive of metastatic disease. She opted for palliative radiotherapy for the primary thyroid cancer and made remarkable postoperative improvement. The authors conclude that surgical treatment of solitary metastatic lesion may produce good symptomatic relief irrespective of patient's age and primary pathology, while emphasising the need for detailed clinical evaluation of patients with 'red flag' symptoms.

  9. Spinal cord compression as initial presentation of metastatic occult follicular thyroid carcinoma.

    Science.gov (United States)

    Khan, Md Nuruzzaman; Sharfuzzaman, Amsm; Mostafa, Md Golam

    2014-04-01

    Metastatic tumors are the most common tumors of the spine, accounting for 98% of all spine lesions. But spinal cord compression as the initial presentation of metastatic occult follicular carcinoma without any thyroid enlargement is unusual and relatively rare. This report describes a 35-years-old female patient presenting with paraplegia and urinary incontinence for the last two months. She had no thyroid enlargement; no thyroid related symptoms and her biochemical thyroid profile was normal. Magnetic resonance imaging (MRI) of spine shows a huge mass compressing the spinal cord at D11-D12 involving both the spinal and paraspinal areas. The patient was treated by surgery and radioiodine ablation as the histopathology showed metastatic follicular thyroid carcinoma. This case was reported because of the rarity of the disease. Early diagnosis and initiation of the treatment should promise a good prognosis for a patient with metastatic spinal cord compression.

  10. Spinal cord compression as initial presentation of metastatic occult follicular thyroid carcinoma

    Directory of Open Access Journals (Sweden)

    Md Nuruzzaman Khan

    2014-01-01

    Full Text Available Metastatic tumors are the most common tumors of the spine, accounting for 98% of all spine lesions. But spinal cord compression as the initial presentation of metastatic occult follicular carcinoma without any thyroid enlargement is unusual and relatively rare. This report describes a 35-years-old female patient presenting with paraplegia and urinary incontinence for the last two months. She had no thyroid enlargement; no thyroid related symptoms and her biochemical thyroid profile was normal. Magnetic resonance imaging (MRI of spine shows a huge mass compressing the spinal cord at D11-D12 involving both the spinal and paraspinal areas. The patient was treated by surgery and radioiodine ablation as the histopathology showed metastatic follicular thyroid carcinoma. This case was reported because of the rarity of the disease. Early diagnosis and initiation of the treatment should promise a good prognosis for a patient with metastatic spinal cord compression.

  11. Transformation of Follicular Lymphoma to a High-Grade B-Cell Lymphoma With MYC and BCL2 Translocations and Overlapping Features of Burkitt Lymphoma and Acute Lymphoblastic Leukemia: A Case Report and Literature Review

    Directory of Open Access Journals (Sweden)

    Alina M Bischin

    2017-02-01

    Full Text Available Most commonly, histologic transformation (HT from follicular lymphoma (FL manifests as a diffuse large B-cell lymphoma, not otherwise specified (DLBCL, NOS. Less frequently, HT may result in a high-grade B-cell lymphoma (HGBL with MYC and B-cell lymphoma protein 2 (BCL2 and/or BCL6 gene rearrangements, also known as “double-hit” or “triple-hit” lymphomas. In the 2016 revision of the World Health Organization (WHO classification of lymphoid neoplasms, the category B-cell lymphoma, unclassifiable was eliminated due to its vague criteria and limiting diagnostic benefit. Instead, the WHO introduced the HGBL category, characterized by MYC and BCL2 and/or BCL6 rearrangements. Cases that present as an intermediate phenotype of DLBCL and Burkitt lymphoma (BL will fall within this HGBL category. Very rarely, HT results in both the intermediate DLBCL and BL phenotypes and exhibits lymphoblastic features, in which case the WHO recommends that this morphologic appearance should be noted. In comparison with de novo patients with DLBCL, NOS, those with MYC and BCL2 and/or BCL6 gene rearrangements have a worse prognosis. A 63-year-old woman presented with left neck adenopathy. Laboratory assessments, including complete blood count, complete metabolic panel, serum lactate dehydrogenase, and β 2 -microglobulin, were all normal. A whole-body computerized tomographic (CT scan revealed diffuse adenopathy above and below the diaphragm. An excisional node biopsy showed grade 3A nodular FL. The Ki67 labeling index was 40% to 50%. A bone marrow biopsy showed a small focus of paratrabecular CD20+ lymphoid aggregates. She received 6 cycles of bendamustine (90 mg/m 2 on days +1 and +2 and rituximab (375 mg/m 2 on day +2, with each cycle delivered every 4 weeks. A follow-up CT scan at completion of therapy showed a partial response with resolution of axillary adenopathy and a dramatic shrinkage of the large retroperitoneal nodes. After 18 months, she had crampy

  12. Neuroendocrine tumor presenting like lymphoma: a case report

    Directory of Open Access Journals (Sweden)

    Vincenzi Bruno

    2011-10-01

    Full Text Available Abstract Introduction Neuroendocrine tumors are a rare but diverse group of malignancies that arise in a wide range of organ systems, including the mediastinum. Differential diagnosis includes other masses arising in the middle mediastinum such as lymphoma, pericardial, bronchogenic and enteric cysts, metastatic tumors, xanthogranuloma, systemic granuloma, diaphragmatic hernia, meningocele and paravertebral abscess. Case presentation We present a case of 42-year-old Caucasian man with a neuroendocrine tumor of the middle-posterior mediastinum and liver metastases, which resembled a lymphoma on magnetic resonance imaging. Conclusion The differential diagnosis in patients with mediastinal masses and liver lesions should include neuroendocrine tumor.

  13. An uncommon presentation of follicular thyroid carcinoma: when chronic back pain should raise a flag.

    Science.gov (United States)

    Alvarado, Milliette; Ramirez-Vick, Margarita; Lopez, Liurka; Marcos-Martinez, Maria J; Saavedra, Fanor M; Negron-Rivera, Juan C; Agosto, Marielba; Martinez, Meliza; Gonzalez, Rafael; Allende-Vigo, Myriam

    2014-01-01

    Follicular thyroid carcinoma is the second most common type of thyroid cancer, and its incidence has increased dramatically in recent years. Although it typically presents as a thyroid nodule, it can spread to distant sites via hematogenous dissemination. Bone metastasis is diagnosed clinically in 2%-13% of patients with differentiated thyroid cancer; nevertheless spinal cord compression complicating thyroid carcinoma is rare and only few cases has been reported in the literature. This case illustrates a strange case of a minimally invasive follicular carcinoma that showed an aggressive behavior, and thus the importance of considering metastatic thyroid carcinoma in the differential diagnosis of chronic back pain progressing to spinal cord compression carrying a severe morbidity.

  14. Safety and Tolerability Study of PCI-32765 in B Cell Lymphoma and Chronic Lymphocytic Leukemia

    Science.gov (United States)

    2017-10-09

    B-cell Chronic Lymphocytic Leukemia; Small Lymphocytic Lymphoma; Diffuse Well-differentiated Lymphocytic Lymphoma; B Cell Lymphoma; Follicular Lymphoma; Mantle Cell Lymphoma; Non-Hodgkin's Lymphoma; Waldenstrom Macroglobulinemia; Burkitt Lymphoma; B-Cell Diffuse Lymphoma

  15. CD57+ T-cells are a subpopulation of T-follicular helper cells in nodular lymphocyte predominant Hodgkin lymphoma

    NARCIS (Netherlands)

    Sattarzadeh, Ahmad; Diepstra, Arjan; Rutgers, Bea; van den Berg, Anke; Visser, Lydia

    2015-01-01

    BACKGROUND: Nodular lymphocyte predominant Hodgkin lymphoma (NLPHL) is characterized by lymphocyte-predominant (LP) cells in a background of CD4+ CD57+ T-cells. These cells are normally present in the germinal center of lymphoid tissues. The cells rosetting LP cells are described to be PD-1 and

  16. CD57+ T-cells are a subpopulation of T-follicular helper cells in nodular lymphocyte predominant Hodgkin lymphoma

    NARCIS (Netherlands)

    Sattarzadeh, Ahmad; Diepstra, Arjan; Rutgers, Bea; van den Berg, Anke; Visser, Lydia

    2015-01-01

    BACKGROUND: Nodular lymphocyte predominant Hodgkin lymphoma (NLPHL) is characterized by lymphocyte-predominant (LP) cells in a background of CD4+ CD57+ T-cells. These cells are normally present in the germinal center of lymphoid tissues. The cells rosetting LP cells are described to be PD-1 and BCL-

  17. Follicular and scarring disorders in skin of color: presentation and management.

    Science.gov (United States)

    Madu, Pamela; Kundu, Roopal V

    2014-08-01

    Skin of color, also known as ethnic skin, is described as skin of individuals of African, Asian, Hispanic, Native-American, Middle Eastern, and Pacific Island backgrounds. Differences in hair morphology, hair grooming, cultural practices, and susceptibility to keloid scarring exist within these populations and have been implicated in hair, scalp, and skin disorders. Acne keloidalis (AK), central centrifugal cicatricial alopecia (CCCA), dissecting cellulitis of the scalp (DCS), pseudofolliculitis barbae (PFB), traction alopecia (TA), and keloids are the most prevalent follicular and scarring disorders in skin of color. They have been associated with disfigurement, permanent hair loss, emotional distress, and decreased quality of life. Hair grooming practices, such as the use of chemical relaxers, heat straightening, and tight braiding and weaving can cause scalp irritation and follicular damage and are linked to the pathogenesis of some of these conditions. Consequently, patient education and behavior modifications are integral to the prevention and management of these disorders. Scarring disorders are also of concern in ethnic populations. Keloid scarring is more prevalent in individuals of African, Asian, and Hispanic descent. The scarring alopecia CCCA is almost exclusively seen in patients of African descent. Therapeutic regimens such as intralesional corticosteroids, surgical excision, and laser therapy can be effective for these follicular and scarring disorders, but carry a risk of dyspigmentation and keloid scarring. Ethnic skin and hair may present unique challenges to the clinician, and knowledge of these differences is essential to providing quality care.

  18. Clinical Safety and Immunogenicity of Tumor-Targeted, Plant-Made Id-KLH Conjugate Vaccines for Follicular Lymphoma

    Directory of Open Access Journals (Sweden)

    Daniel Tusé

    2015-01-01

    Full Text Available We report the first evaluation of plant-made conjugate vaccines for targeted treatment of B-cell follicular lymphoma (FL in a Phase I safety and immunogenicity clinical study. Each recombinant personalized immunogen consisted of a tumor-derived, plant-produced idiotypic antibody (Ab hybrid comprising the hypervariable regions of the tumor-associated light and heavy Ab chains, genetically grafted onto a common human IgG1 scaffold. Each immunogen was produced in Nicotiana benthamiana plants using twin magnICON vectors expressing the light and heavy chains of the idiotypic Ab. Each purified Ab was chemically linked to the carrier protein keyhole limpet hemocyanin (KLH to form a conjugate vaccine. The vaccines were administered to FL patients over a series of ≥6 subcutaneous injections in conjunction with the adjuvant Leukine (GM-CSF. The 27 patients enrolled in the study had previously received non-anti-CD20 cytoreductive therapy followed by ≥4 months of immune recovery prior to first vaccination. Of 11 patients who became evaluable at study conclusion, 82% (9/11 displayed a vaccine-induced, idiotype-specific cellular and/or humoral immune response. No patients showed serious adverse events (SAE related to vaccination. The fully scalable plant-based manufacturing process yields safe and immunogenic personalized FL vaccines that can be produced within weeks of obtaining patient biopsies.

  19. General joint frailty model for recurrent event data with a dependent terminal event: Application to follicular lymphoma data.

    Science.gov (United States)

    Mazroui, Yassin; Mathoulin-Pelissier, Simone; Soubeyran, Pierre; Rondeau, Virginie

    2012-05-20

    Many biomedical studies focus on delaying disease relapses and on prolonging survival. Usual methods only consider one event, often the first recurrence or death. However, ignoring the other recurrences may lead to biased results. The whole history of the disease should be considered for each patient. In addition, some diseases involve recurrences that can increase the risk of death. In this case, the death time may be dependent on the recurrent event history. We propose a joint frailty model to analyze recurrences and death simultaneously. Two gamma-distributed frailties take into account both the inter-recurrences dependence and the dependence between the recurrences and the survival times. We estimate separate parameters for disease recurrent event times and survival times in the joint frailty model to distinguish treatment effects and prognostic factors on these two types of events. We show how maximum penalized likelihood estimation can be applied to semiparametric estimation of the continuous hazard functions in the proposed joint frailty model with right censoring. We also propose parametrical approach. We evaluate the model by simulation studies and illustrate through a study of patients with follicular lymphoma.

  20. Is There a Role for Minimal Residual Disease Monitoring in Follicular Lymphoma in the Chemo-Immunotherapy Era?

    Science.gov (United States)

    Gritti, Giuseppe; Pavoni, Chiara; Rambaldi, Alessandro

    2017-01-01

    After 25 years, evaluation of minimal residual disease (MRD) in follicular lymphoma (FL) has become a standardized technique frequently integrated into clinical trials for its consistent and independent prognostic significance. Achievement of a sustained MRD negativity is a marker of treatment sensibility that has been associated with excellent clinical outcome in terms of clinical response and progression-free survival, independently from the employed therapy. However, no survival advantages has been reported for MRD negative patients and despite the compelling results of clinical trials, MRD evaluation has currently no role in clinical practice. Ongoing clinical trials will help in clarifying the potential setting in which MRD monitoring may have a routine clinical application i.e. allowing de-escalation of standard maintenance therapy in very low risk patients. In this review the clinical implications of MRD monitoring in Rituximab-era are discussed in light of the current treatment paradigms most aimed at reducing toxicities, and the response definition that now routinely integrates PET scan. PMID:28101314

  1. 506U78 in Treating Patients With Recurrent or Refractory Non-Hodgkin's Lymphoma or T-cell Lymphoma

    Science.gov (United States)

    2013-01-22

    Angioimmunoblastic T-cell Lymphoma; Extranodal Marginal Zone B-cell Lymphoma of Mucosa-associated Lymphoid Tissue; Nodal Marginal Zone B-cell Lymphoma; Recurrent Adult T-cell Leukemia/Lymphoma; Recurrent Grade 1 Follicular Lymphoma; Recurrent Grade 2 Follicular Lymphoma; Recurrent Marginal Zone Lymphoma; Recurrent Small Lymphocytic Lymphoma; Splenic Marginal Zone Lymphoma; Waldenström Macroglobulinemia

  2. Research progress on new agents for follicular lymphoma%滤泡性淋巴瘤新药研究进展

    Institute of Scientific and Technical Information of China (English)

    高珊

    2011-01-01

    Follicular lymphoma (FL) is a type of B lymphocytic non-Hodgkin lymphoma.Clinically,FL presents with long course,indolent behavior,and is characterized by prompt response to initial therapy but almost invariably subsequent relapses.It is considered incurable with conventional therapies.Appropriate treatment strategies are critical for survival.Recently,several drugs such as rituximab,and bendamustine have been approved for FL treatment.Very promising novel agents like ofatumumab,epratuzumab,lenalidomide,bortezomib and ABT-263 are now under investigation in clinical trials and will provide more choices for FL therapy.%滤泡性淋巴瘤(FL)是一种起源于B淋巴细胞的非霍奇金淋巴瘤( NHL),临床自然病程较长,表现惰性,初始治疗后易复发,传统的治疗方案多难以治愈.因此选择合适的治疗方案是延长患者生存时间的关键.利妥昔单抗、放射免疫治疗、苯达莫司汀等已被应用于FL的治疗.一些具有前景的新药如ofatumumab、依帕珠单抗(epratuzumab)、雷利度胺、硼替佐米、ABT-263等目前正在进行临床试验,这些新药将会为FL的治疗增加更多的选择.

  3. Lichenoid mucocutaneous syndrome a variant of para neoplastic pemphigus (PNP following the treatment of follicular non-Hodgkin’s lymphoma with fludarabine

    Directory of Open Access Journals (Sweden)

    Katz J

    2013-05-01

    Full Text Available Background: Paraneoplastic pemphigus (PNP is an autoimmune mucocutaneous disease associated with cancer. Since the original description of the condition, various publications have suggested the presence of a heterogeneous spectrum of paraneoplastic mucocutaneous conditions with clinical features of lichenplanus. Several cases of PNP have been reported following treatment with fludarabine. Methods: We present a case of lichenoid syndrome in a follicular B-cell non-Hodgkin lymphoma (NHL patient after treatment with fludarabine and review 8 additional published cases of fludarabine related PNP. Results: Our case is unique due to the fact that the patient presented with lichenoid features both clinically and microscopically and responded well to rituximab therapy. According to literature, both skin and mucosa (eyes and gastrointestinal tract are involved and symptoms start about 1-2 weeks after exposure to fludarabine. Various immunosuppressive treatments have been employed including high dose steroids. Many of these patients developed complications related to the immunosuppressive therapy such as cytomegalovirus, candidiasis and pneumocystis carinii infection and died from respiratory failure. On the other hand, long-term remissions have also been described. Conclusion: Our case represents an unusual case of fludarabine related to mucocutaneous lichenoid syndrome, a variant of PNP, and in view of the outcome in previously described cases, rituximab may be considered a preferred and safe first line therapy for such complication.

  4. Cystic acantholytic dyskeratosis of the vulva: An unusual presentation of a follicular adnexal neoplasm

    Directory of Open Access Journals (Sweden)

    Kara Melissa Tiangco Torres

    2016-01-01

    Full Text Available Acantholytic dyskeratosis (AD is a histologic pattern seen in Darier's disease or dyskeratosis follicularis, warty dyskeratoma, and transient AD. This pattern is characterized by suprabasilar clefting, acantholysis, and formation of corps ronds and grains. We present a case of AD that is unique based on its genital location and cystic architecture. A 53-year-old woman presented with an otherwise asymptomatic cyst on her left vulva of uncertain duration. On microscopic examination, there were fragments of cystic epithelium with areas of hypergranulosis, acantholysis, corps ronds, and corps grains formation. These features are felt by the authors to be a unique presentation of a follicular adnexal neoplasm.

  5. Cardiac Tamponade as Initial Presentation of Hodgkin Lymphoma

    Directory of Open Access Journals (Sweden)

    Adrija Hajra

    2015-01-01

    Full Text Available Cardiac involvement in malignant lymphoma is one of the least investigated subjects. Pericardial effusion is rarely symptomatic in patients of Hodgkin lymphoma (HL. Few case reports are available in the literature. There are case reports of diagnosed HL patients presenting with pericardial effusion. HL patients who present with recurrent episodes of pericardial effusion have also been reported. Pericardial effusion has also been reported in cases of non HL. However, pericardial effusion leading to cardiac tamponade as an initial presentation of HL is extremely rare. Very few such cases are there in the literature. Here, we present a case of a 26-year-old male patient who presented with cardiac tamponade and in due course was found to be a case of classical type of HL. This case is interesting because of its presentation.

  6. An MDM2 antagonist (MI-319 restores p53 functions and increases the life span of orally treated follicular lymphoma bearing animals

    Directory of Open Access Journals (Sweden)

    Yang Dajun

    2009-12-01

    Full Text Available Abstract Background MI-319 is a synthetic small molecule designed to target the MDM2-P53 interaction. It is closely related to MDM2 antagonists MI-219 and Nutlin-3 in terms of the expected working mechanisms. The purpose of this study was to evaluate anti-lymphoma activity of MI-319 in WSU-FSCCL, a B-cell follicular lymphoma line. For comparison purpose, MI-319, MI-219 and Nutlin-3 were assessed side by side against FSCCL and three other B-cell hematological tumor cell lines in growth inhibition and gene expression profiling experiments. Results MI-319 was shown to bind to MDM2 protein with an affinity slightly higher than that of MI-219 and Nutlin-3. Nevertheless, cell growth inhibition and gene expression profiling experiments revealed that the three compounds have quite similar potency against the tumor cell lines tested in this study. In vitro, MI-319 exhibited the strongest anti-proliferation activity against FSCCL and four patient cells, which all have wild-type p53. Data obtained from Western blotting, cell cycle and apoptosis analysis experiments indicated that FSCCL exhibited strong cell cycle arrest and significant apoptotic cell death; cells with mutant p53 did not show significant apoptotic cell death with drug concentrations up to 10 μM, but displayed weaker and differential cell cycle responses. In our systemic mouse model for FSCCL, MI-319 was tolerated well by the animals, displayed effectiveness against FSCCL-lymphoma cells in blood, brain and bone marrow, and achieved significant therapeutic impact (p 28% (%ILS, 14.4 days increase in median survival days. Conclusion Overall, MI-319 probably has an anti-lymphoma potency equal to that of MI-219 and Nutlin-3. It is a potent agent against FSCCL in vitro and in vivo and holds the promises to be developed further for the treatment of follicular lymphoma that retains wild-type p53.

  7. The prognostic value of clonal heterogeneity and quantitative assessment of plasma circulating clonal IG-VDJ sequences at diagnosis in patients with follicular lymphoma

    Science.gov (United States)

    Sarkozy, Clémentine; Huet, Sarah; Carlton, Victoria E.H; Fabiani, Bettina; Delmer, Alain; Jardin, Fabrice; Delfau-Larue, Marie-Helene; Hacini, Maya; Ribrag, Vincent; Guidez, Stéphanie; Faham, Malek; Salles, Gilles

    2017-01-01

    Recent advances in next-generation sequencing (NGS) have enabled the quantitation of circulating tumour DNA (ctDNA) encoding the clonal rearranged V(D)J immunoglobulin locus. We aimed to evaluate the clonal heterogeneity of follicular lymphoma (FL) in the tumour and the plasma at diagnosis and to assess the prognostic value of the ctDNA level. Plasma samples at diagnosis were available for 34 patients registered in the PRIMA trial (NCT00140582). One tumour clonotype or more could be detected for 29 (85%) and 25 (74%) patients, respectively, in the tumour or plasma samples. In 18 patients, several subclones were detected in the tumour (2 to 71 subclones/cases) and/or in the plasma (2 to 20 subclones/cases). In more than half of the cases, the distribution of subclones differed between the tumour and plasma samples, reflecting high clonal heterogeneity and diversity in lymphoma subclone dissemination. In multivariate analysis, a high level of ctDNA was the only independent factor associated with patients’ progression-free survival (HR 4, IC 95 (1.1-37), p=.039). In conclusion, an NGS-based immunosequencing method reveals the marked clonal heterogeneity of follicular lymphoma in patients with FL, and quantification of ctDNA at diagnosis represents a potential powerful prognostic biomarker that needs to be investigated in larger cohorts. PMID:28060738

  8. Coexistence of age-related EBV-associated follicular hyperplasia and large B-cell EBV+ lymphoma of the elderly. Two distinct features of the same T-cell dysfunction related to senescence?

    Science.gov (United States)

    Gibier, Jean-Baptiste; Bouchindhomme, Brigitte; Dubois, Romain; Hivert, Benedicte; Grardel, Nathalie; Copin, Marie-Christine

    2017-03-01

    Age-related EBV-associated lymphoproliferative disorders form a new clinicopathological group. Until recently, this group was associated with diffuse large B-cell lymphoma (DLBCL), characterised by an aggressive clinical presentation and a poor prognosis. Recent findings in Western Caucasian patients, however, suggest that this entity covers a wide spectrum of diseases, ranging from reactive follicular hyperplasia (HR) to DLBCL. We report the case of an 85-year-old Caucasian man showing lymphadenopathy and numerous hypodense lesions of the liver. Examination of a lymph node revealed follicular hyperplasia with EBV expression confined to germinal centres. The patient was treated with Rituximab and subsequently, the lesions of the liver were explored. They showed extensive necrosis and a polymorphic large B-cell population with strong EBV expression. This is the first report to describe age-related EBV-associated follicular hyperplasia at one site coexisting with DLBCL at another. This case warrants undertaking further investigations each time a diagnosis of age-related EBV-HR is associated with extranodal lesions. Copyright © 2016 Elsevier GmbH. All rights reserved.

  9. Genome-wide DNA methylation maps in follicular lymphoma cells determined by methylation-enriched bisulfite sequencing.

    Directory of Open Access Journals (Sweden)

    Jeong-Hyeon Choi

    Full Text Available BACKGROUND: Follicular lymphoma (FL is a form of non-Hodgkin's lymphoma (NHL that arises from germinal center (GC B-cells. Despite the significant advances in immunotherapy, FL is still not curable. Beyond transcriptional profiling and genomics datasets, there currently is no epigenome-scale dataset or integrative biology approach that can adequately model this disease and therefore identify novel mechanisms and targets for successful prevention and treatment of FL. METHODOLOGY/PRINCIPAL FINDINGS: We performed methylation-enriched genome-wide bisulfite sequencing of FL cells and normal CD19(+ B-cells using 454 sequencing technology. The methylated DNA fragments were enriched with methyl-binding proteins, treated with bisulfite, and sequenced using the Roche-454 GS FLX sequencer. The total number of bases covered in the human genome was 18.2 and 49.3 million including 726,003 and 1.3 million CpGs in FL and CD19(+ B-cells, respectively. 11,971 and 7,882 methylated regions of interest (MRIs were identified respectively. The genome-wide distribution of these MRIs displayed significant differences between FL and normal B-cells. A reverse trend in the distribution of MRIs between the promoter and the gene body was observed in FL and CD19(+ B-cells. The MRIs identified in FL cells also correlated well with transcriptomic data and ChIP-on-Chip analyses of genome-wide histone modifications such as tri-methyl-H3K27, and tri-methyl-H3K4, indicating a concerted epigenetic alteration in FL cells. CONCLUSIONS/SIGNIFICANCE: This study is the first to provide a large scale and comprehensive analysis of the DNA methylation sequence composition and distribution in the FL epigenome. These integrated approaches have led to the discovery of novel and frequent targets of aberrant epigenetic alterations. The genome-wide bisulfite sequencing approach developed here can be a useful tool for profiling DNA methylation in clinical samples.

  10. Obinutuzumab (GA101) plus CHOP or FC in relapsed/refractory follicular lymphoma: results of the GAUDI study (BO21000).

    Science.gov (United States)

    Radford, John; Davies, Andrew; Cartron, Guillaume; Morschhauser, Franck; Salles, Gilles; Marcus, Robert; Wenger, Michael; Lei, Guiyuan; Wassner-Fritsch, Elisabeth; Vitolo, Umberto

    2013-08-15

    The safety and activity of obinutuzumab (GA101) plus chemotherapy in relapsed/refractory follicular lymphoma was explored in 56 patients. Participants received obinutuzumab plus cyclophosphamide, doxorubicin, vincristine, and prednisone (G-CHOP; every 3 weeks for 6 to 8 cycles) or obinutuzumab plus fludarabine and cyclophosphamide (G-FC; every 4 weeks for 4 to 6 cycles). Patients were randomly assigned to either obinutuzumab 1600 mg on days 1 and 8 of cycle 1 followed by 800 mg on day 1 of subsequent cycles or 400 mg for all doses. Treatment responders were eligible for obinutuzumab maintenance every 3 months for up to 2 years. Grade 1/2 infusion-related reactions (IRRs) were the most common treatment-related adverse event (AE) (all grades: G-CHOP, 68%; G-FC, 82%). Grade 3/4 IRRs were rare (7%) and restricted to the first infusion. All patients received the planned obinutuzumab dose. Neutropenia was the most common treatment-related hematologic AE for G-CHOP (43%) and G-FC (50%). At induction end, 96% (27/28) of patients receiving G-CHOP (complete response [CR], 39% [11/28]) and 93% (26/28) receiving G-FC (CR, 50% [14 of 28]) achieved responses. G-CHOP and G-FC had an acceptable safety profile with no new or unexpected AEs, but G-FC was associated with more AEs than G-CHOP. Obinutuzumab plus chemotherapy resulted in 93% to 96% response rates, supporting phase 3 investigation. This trial was registered at www.clinicaltrials.gov as #NCT00825149.

  11. Long-term survival outcomes of reduced-intensity allogeneic or autologous transplantation in relapsed grade 3 follicular lymphoma

    Science.gov (United States)

    Klyuchnikov, Evgeny; Bacher, Ulrike; Ahn, Kwang Woo; Carreras, Jeanette; Kröger, Nicolaus M.; Hari, Parameswaran N.; Ku, Grace H.; Ayala, Ernesto; Chen, Andy I.; Chen, Yi-Bin; Cohen, Jonathon B.; Freytes, César O.; Gale, Robert Peter; Kamble, Rammurti T.; Kharfan-Dabaja, Mohamed A.; Lazarus, Hillard M.; Martino, Rodrigo; Mussetti, Alberto; Savani, Bipin N.; Schouten, Harry C.; Usmani, Saad Z.; Wiernik, Peter H.; Wirk, Baldeep; Smith, Sonali M.; Sureda, Anna; Hamadani, Mehdi

    2015-01-01

    Grade-3 follicular lymphoma (FL) has aggressive clinical behavior. To evaluate the optimal first transplantation approach in relapsed/refractory grade-3 FL patients, we compared the long-term outcomes after allogeneic (allo-) vs. autologous hematopoietic cell transplantation (auto-HCT) in the rituximab-era. A total of 197 patients undergoing first RIC allo-HCT or first auto-HCT during 2000-2012 were included. Rituximab-naïve patients were excluded. Allo-HCT recipients were younger; more heavily pretreated, and had a longer interval between diagnosis and HCT. The 5-year probabilities of non-relapse mortality (NRM), relapse/progression, progression-free survival (PFS) and overall survival (OS) for auto-HCT vs. allo-HCT groups were 4% vs. 27% (p<0.001); 61% vs. 20% (p<0.001); 36% vs. 51% (p=0.07) and 59% vs. 54% (p=0.7), respectively. On multivariate analysis auto-HCT was associated with reduced risk of NRM (RR=0.20; p=0.001). Within the first 11months post-HCT auto- and allo-HCT had similar risks of relapse/progression and PFS. Beyond 11months, auto-HCT was associated with higher risk of relapse/progression (RR=21.3; p=0.003) and inferior PFS (RR=3.2; p=0.005). In the first 24 months post-HCT, auto-HCT was associated with improved OS (RR=0.42; p=0.005), but in long-time survivors (beyond 24 months) it was associated with inferior OS (RR=3.6; p=0.04). RIC allo-HCT as the first transplant approach can provide improved PFS and OS, in long-term survivors. PMID:26437062

  12. Genetically Engineered Lymphocyte Therapy After Peripheral Blood Stem Cell Transplant in Treating Patients With High-Risk, Intermediate-Grade, B-cell Non-Hodgkin Lymphoma

    Science.gov (United States)

    2016-08-10

    Recurrent Adult Diffuse Large Cell Lymphoma; Recurrent Adult Diffuse Mixed Cell Lymphoma; Recurrent Adult Diffuse Small Cleaved Cell Lymphoma; Recurrent Grade 3 Follicular Lymphoma; Recurrent Mantle Cell Lymphoma

  13. Targeting malignant B cells as antigen-presenting cells: TLR-9 agonist induces systemic regression of lymphoma.

    Science.gov (United States)

    Klein-González, Nela; Holtick, Udo; Fairfax, Kirsten; Weihrauch, Martin R; von Bergwelt-Baildon, Michael S

    2011-03-01

    Evaluation of: Brody JD, Ai WZ, Czerwinski DK et al. In situ vaccination with a TLR9 agonist induces systemic lymphoma regression: a Phase I/II study. J. Clin. Oncol. 28(28), 4324-4332 (2010). Despite high response rates of the follicular B-cell lymphoma to monoclonal antibodies, the clinical course of lymphoma is still characterized by a continuous pattern of relapse. Brody and colleagues treated 15 patients with relapsed, low-grade lymphoma using low-dose radiotherapy applied to one of the tumor sites with combined injection of a TLR-9 agonist at the same site. This strategy induced specific immunity and tumor regression in several patients with an objective response rate of 27%. The results indicate an involvement of the tumor TLR-9-expressing B cells acting as antigen-presenting cells. TLR-9 in situ vaccination combined with local radiotherapy clearly warrants further in-depth analysis and investigation in a Phase III randomized trial, and may provide a new opportunity for the treatment of B-cell malignancies.

  14. Bilateral primary adrenal lymphoma presenting with adrenal insufficiency

    DEFF Research Database (Denmark)

    Holm, Jakob; Breum, Leif; Stenfeldt, Katrine

    2012-01-01

    surgery was performed. A new computerized tomography scan showed rapid progression of disease with further enlargement of the adrenal masses and both pulmonary and hepatic metastasis. Needle biopsy was performed but the patient refused further treatment and died before a diagnosis was obtained....... The immuneohistochemical diagnosis was large B-cell lymphoma. This case should remind clinicians that PAL may be a cause of bilateral adrenal incidentaloma especially if the patient presents with adrenal insufficiency....

  15. A case of lymphoma presented with acute renal failure

    Directory of Open Access Journals (Sweden)

    Mustafa Yaprak

    2017-03-01

    Full Text Available Acute renal failure (ARF in patients with malignancy occurs due to causes such as prerenal, renal and post renal as in normal population. Tumor infiltration of kidneys is usually uncommon. However, renal function may be impaired in fast-growing hematological malignancies such as acute leukemia or lymphoma, depending on tumor involvement. Herein, we presented a case of ARF and later diagnosed as B-cell Non-Hodgkin's lymphoma. 54-year-old male patient was admitted due to ARF. Although development of ARF due to tumor infiltration is rare, in cases who did not have risk factors for development of ARF, leukemic or lymphomatous infiltration should be considered. [Cukurova Med J 2017; 42(1.000: 168-171

  16. Testicular non-Hodgkin's lymphoma presenting in a young adult

    Science.gov (United States)

    Ratkal, Vishal; Chawla, Arun; Mishra, Dilip Kumar; Monappa, Vidya

    2015-01-01

    We report a case of a 27-year-old man who presented with a slowly growing left testicular swelling associated with mild pain over a period of 3 months. He was evaluated by his family physician with scrotal ultrasound and testicular tumour markers. He was diagnosed and treated as epididymo-orchitis and managed with antibiotics. When he later presented to us, he had an enlarged left testis with normal spermatic cord. Scrotal Doppler evaluation showed a globally enlarged left testis and epididymis with increased vascularity in the left testis, with the right testis being normal. Testicular tumour markers were normal. Fine-needle aspiration cytology of the left testis was suggestive of lymphoma. Exploration through an inguinal approach was carried out and a Chevassu manoeuvre with frozen section study was performed, which was reported as non-Hodgkin's lymphoma. Left radical orchidectomy was performed. Histopathology reported diffuse large B-cell lymphoma, of a germinal centre type. Contrast CT of the abdomen, chest and brain were normal. Sperm cryopreservation was carried out. The patient was started on chemotherapy with cyclophosphamide, hydroxydaunorubicin, oncovin, prednisone (CHOP) regime. PMID:25795748

  17. A Case Report of Neurosarcoidosis Presenting as a Lymphoma Mimic

    Science.gov (United States)

    Cameron, Lauren; Syritsyna, Olga

    2016-01-01

    Objective. To describe a unique presentation of neurosarcoidosis. Background. Central nervous system involvement is rare in sarcoidosis. Sarcoidosis can be severe and can be mistaken for systemic lymphoma. Case Description. A 55-year-old right-handed white male with past medical history of obstructive sleep apnea, Raynaud's disease, and Hashimoto's thyroiditis was noted to have cognitive decline over a duration of few weeks and 20 lb weight loss. His neurologic exam (including cranial nerves) was normal except for five-minute recall. Head CT revealed a lacrimal gland mass, confirmed on brain MRI, which was suspicious for lymphoma on brain PET/MRI. Subsequent whole-body FDG PET/CT scan showed multiple enlarged lymph nodes. Bone marrow biopsy was negative. Serum and CSF ACE levels were within normal limits. Supraclavicular lymph node biopsy before steroids therapy was initiated and revealed multiple noncaseating granulomas, diagnostic of “sarcoidosis.” He was treated with daily prednisone for two months, followed by weekly infliximab. Brain MRI two months after treatment with prednisone showed decrease in size of lacrimal lesion, and brain PET/MRI showed normal brain metabolism pattern after five months. Neurocognitive evaluation three months after diagnosis demonstrated improvements in memory abilities. Discussion. Both clinically and radiographically, neurosarcoidosis can mimic systemic lymphoma. Biopsy in these types of cases is necessary to establish the diagnosis. PMID:27800198

  18. MRI findings of malignant lymphoma presenting in bones

    Energy Technology Data Exchange (ETDEWEB)

    Moon, Sungwook; Cho, Jeamin; Suh, Jin Suck [College of Medicine, Yonsei University, Seoul (Korea, Republic of); Ha, Doohoe [CHA General Hospital, College of Medicine, Pochon CHA University, Seoul (Korea, Republic of)

    2000-02-01

    To analyze the MR finding of skeletal malignant lymphoma. The authors retrospectively reviewed the MR imaging findings (location, signal intensity and contrastenhancement of lesions, and patterns of bone marrow involvement) of 12 patients with malignant lymphoma presenting in bones and confirmed by incisional biopsy, blind bone marrow biopsy or follow-up MR imaging. Among the 15 lesions in 12 patients, the spine was the most commonly involved site (n=3D5), followed by the ilium (n=3D4), femur (n=3D3), sternum (n=3D2), titia (n=3D1). On T1-weighted images, lesions showed signal intensity close to muscle, while on T2-weighted images, involved bones showed variable signal intensity from muscle to water signal intensity. Patterns of bone marrow involvement were nodular (n=3D2), diffuse (n=3D5), or segmental (n=3D8). All cases of segmental bone marrow involvement also produced soft tissue mass. In two cases in which this occurred, cortical breakdown was not noted. On T1- and T2-weighted MR images, particularly the latter, bone marrow involvement of malignant lymphoma was observed, with variable signal intensity. Contrast enhanced images revealed distinct lesion. Patterns of bone marrow involvement varied. (author)

  19. A Case Report of Neurosarcoidosis Presenting as a Lymphoma Mimic

    Directory of Open Access Journals (Sweden)

    Gurcharanjeet Kaur

    2016-01-01

    Full Text Available Objective. To describe a unique presentation of neurosarcoidosis. Background. Central nervous system involvement is rare in sarcoidosis. Sarcoidosis can be severe and can be mistaken for systemic lymphoma. Case Description. A 55-year-old right-handed white male with past medical history of obstructive sleep apnea, Raynaud’s disease, and Hashimoto’s thyroiditis was noted to have cognitive decline over a duration of few weeks and 20 lb weight loss. His neurologic exam (including cranial nerves was normal except for five-minute recall. Head CT revealed a lacrimal gland mass, confirmed on brain MRI, which was suspicious for lymphoma on brain PET/MRI. Subsequent whole-body FDG PET/CT scan showed multiple enlarged lymph nodes. Bone marrow biopsy was negative. Serum and CSF ACE levels were within normal limits. Supraclavicular lymph node biopsy before steroids therapy was initiated and revealed multiple noncaseating granulomas, diagnostic of “sarcoidosis.” He was treated with daily prednisone for two months, followed by weekly infliximab. Brain MRI two months after treatment with prednisone showed decrease in size of lacrimal lesion, and brain PET/MRI showed normal brain metabolism pattern after five months. Neurocognitive evaluation three months after diagnosis demonstrated improvements in memory abilities. Discussion. Both clinically and radiographically, neurosarcoidosis can mimic systemic lymphoma. Biopsy in these types of cases is necessary to establish the diagnosis.

  20. Double-Hit Lymphoma Presenting as Primary Renal Lymphoma: A Case Report

    Directory of Open Access Journals (Sweden)

    Vikas Mehta

    2013-04-01

    Full Text Available B-cell lymphomas with concurrent IGH-BCL2 and MYC rearrangements, also known as “double hit” lymphomas (DHL, are rare neoplasms characterized by highly aggressive clinical behavior, complex karyotypes, and a spectrum of pathological features overlapping with Burkitt lymphoma (BL and diffuse large B-cell lymphoma (DLBCL. Primary renal lymphoma (PRL by definition is a renal lymphoma without evidence of systemic involvement. PRL is extremely rare with less than 100 cases of both Hodgkin disease and non-Hodgkin lymphoma reported in literature. Double hit lymphomas have extremely poor prognosis, and high resistance to intensive chemotherapy, including high-dose chemotherapy. We describe a very rare case of DHL arising in kidney as PRL in whom concurrent IGH-BCL2 and MYC rearrangements were detected. [J Interdiscipl Histopathol 2013; 1(2.000: 93-97

  1. Rituximab maintenance improves clinical outcome of relapsed/resistant follicular non-Hodgkin lymphoma in patients both with and without rituximab during induction: results of a prospective randomized phase 3 intergroup trial

    NARCIS (Netherlands)

    M.H.J. van Oers; R. Klasa; R.E. Marcus; M. Wolf; E. Kimby; R.D. Gascoyne; A. Jack; M. van't Veer; A. Vranovsky; H. Holte; M. van Glabbeke; I. Teodorovic; C. Rozewicz; A. Hagenbeek

    2006-01-01

    We evaluated the role of rituximab (R) both in remission induction and maintenance treatment of relapsed/resistant follicular lymphoma (FL). A total of 465 patients were randomized to induction with 6 cycles of cyclophosphamide, doxorubicin, vincristine, and prednisone (CHOP) (every 3 weeks) or R-CH

  2. Protein kinase CK2 is widely expressed in follicular, Burkitt and diffuse large B-cell lymphomas and propels malignant B-cell growth.

    Science.gov (United States)

    Pizzi, Marco; Piazza, Francesco; Agostinelli, Claudio; Fuligni, Fabio; Benvenuti, Pietro; Mandato, Elisa; Casellato, Alessandro; Rugge, Massimo; Semenzato, Gianpietro; Pileri, Stefano A

    2015-03-30

    Serine-threonine kinase CK2 is highly expressed and pivotal for survival and proliferation in multiple myeloma, chronic lymphocytic leukemia and mantle cell lymphoma. Here, we investigated the expression of α catalytic and β regulatory CK2 subunits by immunohistochemistry in 57 follicular (FL), 18 Burkitt (BL), 52 diffuse large B-cell (DLBCL) non-Hodgkin lymphomas (NHL) and in normal reactive follicles. In silico evaluation of available Gene Expression Profile (GEP) data sets from patients and Western blot (WB) analysis in NHL cell-lines were also performed. Moreover, the novel, clinical-grade, ATP-competitive CK2-inhibitor CX-4945 (Silmitasertib) was assayed on lymphoma cells. CK2 was detected in 98.4% of cases with a trend towards a stronger CK2α immunostain in BL compared to FL and DLBCL. No significant differences were observed between Germinal Center B (GCB) and non-GCB DLBCL types. GEP data and WB confirmed elevated CK2 mRNA and protein levels as well as active phosphorylation of specific targets in NHL cells. CX-4945 caused a dose-dependent growth-arresting effect on GCB, non-GCB DLBCL and BL cell-lines and it efficiently shut off phosphorylation of NF-κB RelA and CDC37 on CK2 target sites. Thus, CK2 is highly expressed and could represent a suitable therapeutic target in BL, FL and DLBCL NHL.

  3. Primary skeletal muscle lymphoma presenting as refractory cellulitis.

    Science.gov (United States)

    Baddour, L M; Haden, K H; Allen, J W

    2001-09-01

    The right torso of a 55-year-old woman showed diffuse skin and soft-tissue changes suggestive of cellulitis. However, several clinical and radiologic features, including the subacute and non-toxic nature of the illness and the patient's lack of response to antibiotic therapy, indicated a noninfectious etiology. Malignancy was suggested by striking changes seen on computed tomographic scanning--including extensive infiltration and enlargement of the musculature of the right shoulder girdle, the intercostal musculature, the latissimus dorsi, and the rhomboids; focal enlargement of the right paraspinal muscles; and enlargement of the psoas and the iliacus muscles and of the musculature around the hip joint. The mediastinal, hilar, and paraaortic regions showed no adenopathy. A large hypodense lesion of approximately 4.5 cm, which was seen in the caudate lobe of the liver, raised the concern of a metastatic focus of malignancy. Because of these findings, an immediate muscle biopsy was performed. Results showed a non-Hodgkin's lymphoma with a B-cell phenotype. Although primary skeletal muscle lymphoma is very uncommon in patients without human immunodeficiency virus infection, clinical presentation of refractory cellulitis, as seen in the current case, is extremely rare.

  4. Association of clinical status of follicular lymphoma patients after autologous stem cell transplant and quantitative assessment of lymphoma in blood and bone marrow as measured by SYBR Green I polymerase chain reaction.

    Science.gov (United States)

    Pennell, Nancy; Woods, Anthony; Reis, Marciano; Buckstein, Rena; Spaner, David; Imrie, Kevin; Hewitt, Karen; Boudreau, Angela; Seth, Arun; Berinstein, Neil L

    2006-02-01

    Molecular remission in the autograft and bone marrow after transplant are predictive of durable clinical remission in relapsed follicular lymphoma. Thus, a simple reliable method to quantify minimal residual disease (MRD) would improve prognostication in these patients. Fluorescent hybridization probes have been used in real-time quantitative polymerase chain reaction (RQ-PCR) to monitor MRD with a reproducible sensitivity of 0.01%; however, these techniques are expensive and require additional experiments to examine clonality. We describe a SYBR Green I detection method that is more universal, checks clonal identity, yields the same sensitivity for monitoring MRD, and is more economically attractive. Using this method to follow 14 follicular lymphoma patients treated with autologous stem cell transplantation, molecular markers were successfully defined for 12 patients. Median contamination of stem-cell grafts was 0.1% (range, 0 to 13%). Six patients with measurable graft contamination became PCR-negative in blood and bone marrow within 12 months after autologous stem cell transplantation. Three patients free of disease progression (median follow-up of 75 months) are in molecular remission. Increasing fractions of RQ-PCR-positive blood and bone marrow cells reliably predicted morphological and clinical relapse. In one case, both clinical relapse and spontaneous regression were reflected by changes in MRD levels. Thus, our RQ-PCR method reproducibly distinguishes different levels of MRD.

  5. Interleukin-2 or Observation Following Radiation Therapy, Combination Chemotherapy, and Peripheral Stem Cell Transplantation in Treating Patients With Recurrent Non-Hodgkin's Lymphoma

    Science.gov (United States)

    2013-02-27

    Recurrent Adult Burkitt Lymphoma; Recurrent Adult Diffuse Large Cell Lymphoma; Recurrent Adult Diffuse Mixed Cell Lymphoma; Recurrent Adult Diffuse Small Cleaved Cell Lymphoma; Recurrent Adult Immunoblastic Large Cell Lymphoma; Recurrent Grade 1 Follicular Lymphoma; Recurrent Grade 2 Follicular Lymphoma; Recurrent Grade 3 Follicular Lymphoma

  6. Utility of quantitative FDG-PET/CT for the detection of bone marrow involvement in follicular lymphoma: a histopathological correlation study

    Energy Technology Data Exchange (ETDEWEB)

    Adams, Hugo J.A.; Kwee, Thomas C.; Nievelstein, Rutger A.J. [University Medical Center Utrecht, Department of Radiology and Nuclear Medicine, Utrecht (Netherlands); Fijnheer, Rob [Meander Medical Center, Department of Hematology, Amersfoort (Netherlands); Dubois, Stefan V. [Meander Medical Center, Department of Pathology, Amersfoort (Netherlands); Blase, Peter E.; Klerk, John M.H. de [Meander Medical Center, Department of Nuclear Medicine, Amersfoort (Netherlands)

    2014-09-15

    To determine the value of visual and quantitative {sup 18}F-fluoro-2-deoxy-D-glucose positron emission tomography/computed tomography (FDG-PET/CT) for the detection of bone marrow involvement in follicular lymphoma, using direct histopathological examination at the right posterior iliac crest as reference standard. This retrospective study included 22 patients with newly diagnosed follicular lymphoma who had undergone FDG-PET/CT before BMB of the right posterior iliac crest. FDG-PET/CT images were visually evaluated for bone marrow involvement in the right posterior iliac crest. Volumes of interest were placed in the right posterior iliac crest to calculate the 3D partial volume corrected mean standardized uptake value (cSUV{sub mean}), maximum standardized uptake value (SUV{sub max}) and peak standardized uptake value (SUV{sub peak}). Sensitivity and specificity of visual FDG-PET/CT analysis for the detection of bone marrow involvement in the right posterior iliac crest were 0.0 % (95 % confidence interval (CI): 0-32.4 %) and 100 % (95 % CI: 78.5-100 %), respectively. Areas under the receiver-operating characteristic curve of cSUV{sub mean}, SUV{sub max} and SUV{sub peak} for the detection of bone marrow involvement in the right posterior iliac crest were 0.85 (95 % CI: 0.63-0.96), 0.89 (95 % CI: 0.68-0.98) and 0.87 (95 % CI: 0.65-0.97), respectively. Optimal cutoff values for cSUV{sub mean}, SUV{sub max} and SUV{sub peak} were 1.3, 2.1 and 1.7, and yielded sensitivity and specificity combinations of 75.0 % and 85.7 %, 87.5 % and 85.7 % and 87.5 % and 85.7 %, respectively. This histopathological correlation study shows that, unlike visual interpretation of FDG-PET/CT images, quantitative FDG-PET/CT analysis may be beneficial in diagnosing bone marrow involvement by follicular lymphoma. (orig.)

  7. Genetically Engineered Lymphocytes, Cyclophosphamide, and Aldesleukin in Treating Patients With Relapsed or Refractory Mantle Cell Lymphoma or Indolent B-Cell Non-Hodgkin Lymphoma

    Science.gov (United States)

    2014-08-04

    B-cell Chronic Lymphocytic Leukemia; Extranodal Marginal Zone B-cell Lymphoma of Mucosa-associated Lymphoid Tissue; Nodal Marginal Zone B-cell Lymphoma; Recurrent Grade 1 Follicular Lymphoma; Recurrent Grade 2 Follicular Lymphoma; Recurrent Mantle Cell Lymphoma; Recurrent Marginal Zone Lymphoma; Recurrent Small Lymphocytic Lymphoma; Refractory Chronic Lymphocytic Leukemia; Splenic Marginal Zone Lymphoma; Waldenström Macroglobulinemia

  8. A population-based study of prognosis in advanced stage follicular lymphoma managed by watch and wait

    DEFF Research Database (Denmark)

    El-Galaly, Tarec Christoffer; Bilgrau, Anders E; de Nully Brown, Peter

    2015-01-01

    % (95%CI 7-20). Elevated lactate dehydrogenase and > four nodal regions involved were associated with a higher risk of lymphoma treatment and death from lymphoma. The WAW patients and a matched background population had similar OS during the first 50 months after diagnosis (P = 0·7), but WAW patients...

  9. Everolimus and Lenalidomide in Treating Patients With Relapsed or Refractory Non-Hodgkin or Hodgkin Lymphoma

    Science.gov (United States)

    2016-04-18

    Adult Nasal Type Extranodal NK/T-cell Lymphoma; Anaplastic Large Cell Lymphoma; Angioimmunoblastic T-cell Lymphoma; Extranodal Marginal Zone B-cell Lymphoma of Mucosa-associated Lymphoid Tissue; Hepatosplenic T-cell Lymphoma; Nodal Marginal Zone B-cell Lymphoma; Peripheral T-cell Lymphoma; Post-transplant Lymphoproliferative Disorder; Recurrent Adult Burkitt Lymphoma; Recurrent Adult Diffuse Large Cell Lymphoma; Recurrent Adult Hodgkin Lymphoma; Recurrent Adult T-cell Leukemia/Lymphoma; Recurrent Grade 1 Follicular Lymphoma; Recurrent Grade 2 Follicular Lymphoma; Recurrent Grade 3 Follicular Lymphoma; Recurrent Mantle Cell Lymphoma; Recurrent Mycosis Fungoides/Sezary Syndrome; Splenic Marginal Zone Lymphoma; Waldenstrom Macroglobulinemia

  10. Bryostatin 1 Plus Vincristine in Treating Patients With Progressive or Relapsed Non-Hodgkin's Lymphoma After Bone Marrow or Stem Cell Transplantation

    Science.gov (United States)

    2013-01-09

    Extranodal Marginal Zone B-cell Lymphoma of Mucosa-associated Lymphoid Tissue; Nodal Marginal Zone B-cell Lymphoma; Recurrent Adult Diffuse Large Cell Lymphoma; Recurrent Adult Diffuse Mixed Cell Lymphoma; Recurrent Adult Diffuse Small Cleaved Cell Lymphoma; Recurrent Grade 1 Follicular Lymphoma; Recurrent Grade 2 Follicular Lymphoma; Recurrent Grade 3 Follicular Lymphoma; Recurrent Mantle Cell Lymphoma; Recurrent Marginal Zone Lymphoma; Recurrent Small Lymphocytic Lymphoma; Splenic Marginal Zone Lymphoma

  11. Lenalidomide and Combination Chemotherapy (DA-EPOCH-R) in Treating Patients With MYC-Associated B-Cell Lymphomas

    Science.gov (United States)

    2016-08-24

    Adult Grade III Lymphomatoid Granulomatosis; B-cell Chronic Lymphocytic Leukemia; Contiguous Stage II Adult Diffuse Large Cell Lymphoma; Contiguous Stage II Adult Diffuse Mixed Cell Lymphoma; Contiguous Stage II Adult Diffuse Small Cleaved Cell Lymphoma; Contiguous Stage II Adult Immunoblastic Large Cell Lymphoma; Contiguous Stage II Grade 1 Follicular Lymphoma; Contiguous Stage II Grade 2 Follicular Lymphoma; Contiguous Stage II Grade 3 Follicular Lymphoma; Contiguous Stage II Mantle Cell Lymphoma; Contiguous Stage II Marginal Zone Lymphoma; Contiguous Stage II Small Lymphocytic Lymphoma; Cutaneous B-cell Non-Hodgkin Lymphoma; Extranodal Marginal Zone B-cell Lymphoma of Mucosa-associated Lymphoid Tissue; Intraocular Lymphoma; Nodal Marginal Zone B-cell Lymphoma; Noncontiguous Stage II Adult Diffuse Large Cell Lymphoma; Noncontiguous Stage II Adult Diffuse Mixed Cell Lymphoma; Noncontiguous Stage II Adult Diffuse Small Cleaved Cell Lymphoma; Noncontiguous Stage II Adult Immunoblastic Large Cell Lymphoma; Noncontiguous Stage II Grade 1 Follicular Lymphoma; Noncontiguous Stage II Grade 2 Follicular Lymphoma; Noncontiguous Stage II Grade 3 Follicular Lymphoma; Noncontiguous Stage II Mantle Cell Lymphoma; Noncontiguous Stage II Marginal Zone Lymphoma; Noncontiguous Stage II Small Lymphocytic Lymphoma; Progressive Hairy Cell Leukemia, Initial Treatment; Small Intestine Lymphoma; Splenic Marginal Zone Lymphoma; Stage 0 Chronic Lymphocytic Leukemia; Stage I Adult Diffuse Large Cell Lymphoma; Stage I Adult Diffuse Mixed Cell Lymphoma; Stage I Adult Diffuse Small Cleaved Cell Lymphoma; Stage I Adult Hodgkin Lymphoma; Stage I Adult Immunoblastic Large Cell Lymphoma; Stage I Chronic Lymphocytic Leukemia; Stage I Grade 1 Follicular Lymphoma; Stage I Grade 2 Follicular Lymphoma; Stage I Grade 3 Follicular Lymphoma; Stage I Mantle Cell Lymphoma; Stage I Marginal Zone Lymphoma; Stage I Small Lymphocytic Lymphoma; Stage II Adult Hodgkin Lymphoma; Stage II Chronic Lymphocytic

  12. Primary bone marrow lymphoma: an uncommon extranodal presentation of aggressive non-hodgkin lymphomas.

    NARCIS (Netherlands)

    Martinez, A.; Ponzoni, M.; Agostinelli, C.; Hebeda, K.M.; Matutes, E.; Peccatori, J.; Campidelli, C.; Espinet, B.; Perea, G.; Acevedo, A.; Mehrjardi, A.Z.; Martinez-Bernal, M.; Gelemur, M.; Zucca, E.; Pileri, S.; Campo, E.; Lopez-Guillermo, A.; Rozman, M.

    2012-01-01

    Bone marrow involvement by lymphoma is considered a systemic dissemination of the disease arising elsewhere, although some tumors may arise primarily in the bone marrow microenvironment. Primary bone marrow lymphoma (PBML) is a rare entity whose real boundaries and clinicobiological significance are

  13. Vacuna idiotípica en el tratamiento del linfoma folicular: situación actual y perspectivas futuras Idiotype vaccines in the treatment of follicular lymphoma: current status and future perspectives

    Directory of Open Access Journals (Sweden)

    N. Zabalegui

    2009-04-01

    an indolent disease with a slow progression in which, although exhibiting a good response to treatment, relapse is very frequent and complete remission is not easy to maintain. Therefore, the disease is regarded as incurable. The search for new therapeutic strategies, together with a better understanding of the immune system, has led to the emergence of a new treatment named immunotherapy. Follicular lymphoma is a malignancy suitable for this kind of treatment given the fact that it is characterized by presenting a unique tumour-specific antigen: the idiotype of the monoclonal immunoglobulin displayed on the membrane of tumour cells. Several studies have been conducted to test immunotherapy as complementary to conventional treatment. In a previous study by our group, a clear benefit was evident is obtained after idiotypic vaccination, when an adequate immunization of the patient is obtained, in comparison to chemotherapy alone. In this sense, analysis is needed of whether idiotypic vaccination can produce not only long-lasting and complete remission, but even cure. It would be of great interest to consider an optimisation of the experimental design of clinical trials, an improvement of vaccine production, and the study of the molecular mechanisms of the tumour cell which modify the target immunoglobulin.

  14. Arsenic Trioxide in Treating Patients With Relapsed or Refractory Lymphoma or Leukemia

    Science.gov (United States)

    2013-01-31

    Extranodal Marginal Zone B-cell Lymphoma of Mucosa-associated Lymphoid Tissue; Nodal Marginal Zone B-cell Lymphoma; Prolymphocytic Leukemia; Recurrent Adult Diffuse Small Cleaved Cell Lymphoma; Recurrent Grade 1 Follicular Lymphoma; Recurrent Grade 2 Follicular Lymphoma; Recurrent Marginal Zone Lymphoma; Recurrent Small Lymphocytic Lymphoma; Refractory Chronic Lymphocytic Leukemia; Splenic Marginal Zone Lymphoma; Waldenström Macroglobulinemia

  15. Lymphoma Presenting as Severe Left Ventricular Systolic Dysfunction: A Case Report

    Directory of Open Access Journals (Sweden)

    Imran Hafeez

    2014-05-01

    Full Text Available Lymphoma involving the heart is rare. This is a case report on non-Hodgkin lymphoma where the patient presented for the first time with heart failure and severe left ventricular systolic dysfunction due to lymphoma infiltrating the heart muscle and had simultaneous bilateral involvement of kidneys. This type of presentation has never been described in world literature and is the first reported case.

  16. Chronic lymphocytic leukemia/small lymphocytic lymphoma presenting as septic arthritis of the shoulder

    Energy Technology Data Exchange (ETDEWEB)

    Donovan, Andrea; Schweitzer, Mark E.; Nomikos, George [NYU Hospital for Joint Diseases, New York, NY (United States); Garcia, Roberto A. [Bellevue Hospital Center, New York, NY (United States)

    2008-11-15

    We report a case of a 53-year-old man presenting with shoulder pain mimicking septic arthritis. Laboratory findings were atypical. Biopsy performed to assess for possible osteomyelitis demonstrated chronic lymphocytic leukemia/small lymphocytic lymphoma. Intra-articular lymphoma is a rare but important consideration in patients with atypical clinical presentation. Imaging alone may be insufficient to render diagnosis as lymphoma can mimic infection, synovial hypertrophic processes, and depositional arthropathy. (orig.)

  17. Intravascular large B-cell lymphoma presenting with fulminant pseudomembranous colitis.

    Science.gov (United States)

    Wang, Tao; Ghaffar, Hasan; Grin, Andrea

    2013-05-01

    Intravascular large B-cell lymphoma is a rare entity that usually presents in late stages with non-specific symptoms. We present a case of an incidentally discovered intravascular large B-cell lymphoma in a 78-year-old man who underwent colectomy for medically refractory pseudomembranous colitis. The malignant lymphocytes were preferentially localized to small colonic submucosal vasculature, without any evidence of an extravascular tumor mass. The gastrointestinal system is an exceeding rare initial diagnostic site for intravascular lymphoma, and presentation with pseudomembranous colitis has not been previously reported. We discuss the current definition of intravascular lymphoma, clinicopathological variants, differential diagnoses, as well as current therapy.

  18. Binding of peanut lectin to germinal-centre cells: a marker for B-cell subsets of follicular lymphoma?

    OpenAIRE

    De Rose, M L; Habeshaw, J A; R. Kennedy; Sloane, J.; Wiltshaw, E; Davies, A. J.

    1981-01-01

    The binding of horseradish-peroxidase-labelled peanut lectin (HRP-PNL) to cryostat sections of tonsil, lymphoma lymph nodes, reactive lymph nodes and miscellaneous tumours demonstrated that PNL binds selectively to lymphocytes in germinal centres. Lymph nodes from 21 patients with non-Hodgkin's lymphomas were phenotyped as cell suspensions for PNL binding, and the following surface markers: E rosetting, C3d, SIg, OK markers of T-cell subsets, Ig heavy-chain and light-chain classes. There was ...

  19. Extranodal non-Hodgkin's lymphoma presenting as gingival mass

    Science.gov (United States)

    Manjunatha, B. S.; Gowramma, R.; Nagarajappa, D.; Tanveer, Ahmed

    2011-01-01

    Non-Hodgkin's lymphoma (NHL) commonly presents as non-tender, enlarged lymph nodes, accompanied by diffuse symptoms of fatigue and low-grade intermittent fever and it is derived predominantly from the cells of the B lymphocyte series. NHL cases occur extra-nodally and in 3% of these cases the initial presentation may be in the oral cavity. Though extra-nodal NHL of the oral cavity is a rare finding, patients with oral lesions of NHL commonly present at the dental clinic in the first instance. A careful clinical evaluation supported by histopathological and other laboratory investigations will help in identifying the disease at an early stage, resulting in better prognosis. Any delay in diagnosis has important implications on the morbidity and mortality of the condition. Due to the rarity of intraoral NHL, we present one such a case with a complaint of tumor-like mass on the gingiva of lower molar region. The lesion was clinically thought as pyogenic granuloma and later diagnosed as extra nodal NHL of the oral cavity. PMID:22368372

  20. Cerebral infratentorial large B-cell lymphoma presenting as Parkinsonism.

    Science.gov (United States)

    Lin, Chih-Ming; Hong, Kelvin

    2010-03-01

    Though rare, primary intracranial tumors can present with Parkinsonian symptoms, and diagnosis can be delayed unless there is a high index of suspicion. We herein present an 81-year-old man who was seen in our neurology clinic due to acute onset of unsteady gait and altered consciousness. Parkinsonism was initially diagnosed because of the typical manifestations. Levodopa was prescribed; however, there was a limited effect on his symptoms. Upon detail history and neurological examination, left sided hemiparesis was disclosed. Cerebral imaging studies revealed a solid mass over the right infratentorial para-midbrain area leading to reactive obstructive hydrocephalus. Work-up including chest and abdominal CT scanning, upper and lower GI endoscopy, and tumor marker studies failed to uncover any abnormalities. A neurosurgeon was consulted and a shunt procedure and biopsy of the infratentorial mass were performed. Histopathological examination of the biopsy tissue revealed tumor diffusely intermixed with large cells consistent with large B-cell lymphoma. The patient and his family declined further treatment. Though rare, cerebral tumors can present with Parkinsonian features and represent a diagnostic challenge. Clinicians should be aware of the possibility of cerebral neoplasms causing Parkinsonism, and include them in the differential diagnosis, especially for patients presenting with atypical Parkinsonian features, or those not responsive to initial therapy.

  1. Follicular dermographism.

    Science.gov (United States)

    Shelley, W B; Shelley, E D

    1983-09-01

    Four patients who complained of an inexplicable pruritus of several weeks to several years in duration are presented. General medical studies were unremarkable and the skin was normal except for erythematous papular lesions at sites recently scratched. The skin changes could be reproduced by multiple firm strokes with the broad edge of a tongue blade. The transitory, discrete, follicular, urticarial papules so elicited have been labeled by us as follicular dermographism. It is an entity to be distinguished from cholinergic and aquagenic urticaria. We postulate that follicular dermographism occurs during periods of transient antigenemia. At such times, the frictional force of scratching or the tongue blade test releases antigen from the blood stream to trigger focal urticaria at sites of high density mast cells, namely around the hair follicle. Hydroxyzine is an effective therapy.

  2. An Open-Label, Multicenter, Phase 1/2 Study of E7438 (EZH2 Histone Methyl Transferase [HMT] Inhibitor) as a Single Agent in Subjects With Advanced Solid Tumors or With B-cell Lymphomas

    Science.gov (United States)

    2016-09-01

    B-cell Lymphomas (Phase 1); Advanced Solid Tumors (Phase 1); Diffuse Large B-cell Lymphoma (Phase 2); Follicular Lymphoma (Phase 2); Transformed Follicular Lymphoma; Primary Mediastinal Large B-Cell Lymphoma

  3. Water-only fasting and an exclusively plant foods diet in the management of stage IIIa, low-grade follicular lymphoma.

    Science.gov (United States)

    Goldhamer, Alan C; Klaper, Michael; Foorohar, Afsoon; Myers, Toshia R

    2015-12-10

    Follicular lymphoma (FL), the second most common non-Hodgkin's lymphoma (NHL), is well characterised by a classic histological appearance and an indolent course. Current treatment protocols for FL range from close observation to immunotherapy, chemotherapy and/or radiotherapies. We report the case of a 42-year-old woman diagnosed by excisional biopsy with stage IIIa, grade 1 FL. In addition to close observation, the patient underwent a medically supervised, 21-day water-only fast after which enlarged lymph nodes were substantially reduced in size. The patient then consumed a diet of minimally processed plant foods free of added sugar, oil and salt (SOS), and has remained on the diet since leaving the residential facility. At 6 and 9-month follow-up visits, the patient's lymph nodes were non-palpable and she remained asymptomatic. This case establishes a basis for further studies evaluating water-only fasting and a plant foods, SOS-free diet as a treatment protocol for FL. 2015 BMJ Publishing Group Ltd.

  4. Coding variants at hexa-allelic amino acid 13 of HLA-DRB1 explain independent SNP associations with follicular lymphoma risk.

    Science.gov (United States)

    Foo, Jia Nee; Smedby, Karin E; Akers, Nicholas K; Berglund, Mattias; Irwan, Ishak D; Jia, Xiaoming; Li, Yi; Conde, Lucia; Darabi, Hatef; Bracci, Paige M; Melbye, Mads; Adami, Hans-Olov; Glimelius, Bengt; Khor, Chiea Chuen; Hjalgrim, Henrik; Padyukov, Leonid; Humphreys, Keith; Enblad, Gunilla; Skibola, Christine F; de Bakker, Paul I W; Liu, Jianjun

    2013-07-11

    Non-Hodgkin lymphoma represents a diverse group of blood malignancies, of which follicular lymphoma (FL) is a common subtype. Previous genome-wide association studies (GWASs) have identified in the human leukocyte antigen (HLA) class II region multiple independent SNPs that are significantly associated with FL risk. To dissect these signals and determine whether coding variants in HLA genes are responsible for the associations, we conducted imputation, HLA typing, and sequencing in three independent populations for a total of 689 cases and 2,446 controls. We identified a hexa-allelic amino acid polymorphism at position 13 of the HLA-DR beta chain that showed the strongest association with FL within the major histocompatibility complex (MHC) region (multiallelic p = 2.3 × 10⁻¹⁵). Out of six possible amino acids that occurred at that position within the population, we classified two as high risk (Tyr and Phe), two as low risk (Ser and Arg), and two as moderate risk (His and Gly). There was a 4.2-fold difference in risk (95% confidence interval = 2.9-6.1) between subjects carrying two alleles encoding high-risk amino acids and those carrying two alleles encoding low-risk amino acids (p = 1.01 × 10⁻¹⁴). This coding variant might explain the complex SNP associations identified by GWASs and suggests a common HLA-DR antigen-driven mechanism for the pathogenesis of FL and rheumatoid arthritis.

  5. Burkitt Lymphoma Presenting as Unilateral Deafness in an Immunocompetent Patient

    Directory of Open Access Journals (Sweden)

    Andre Pinto

    2012-01-01

    Full Text Available A 55-year-old HIV-negative white male presented with right ear deafness, right axillary lymphadenopathy, and weight loss. Laboratory findings included anemia, marked leukocytosis, and thrombocytopenia. Examination of the peripheral smear demonstrated the presence of increased circulating blast-like cells of intermediate size, with basophilic cytoplasm and nuclei with open chromatin. MRI of the brain was compatible with hemorrhagic labyrinthitis. Excisional biopsy of the axillary mass revealed an enlarged lymph node with effaced architecture and “starry sky” appearance. The cells expressed CD20, CD10, BCL6, and surface kappa immunoglobulin light chain, with a high proliferative index by immunohistochemistry and flow cytometry. Subsequent bone marrow biopsy was hypercellular (approximately 95%, with blast-like cells virtually replacing all hematopoietic elements. Routine karyotype as well as FISH analysis of bone marrow cells demonstrated rearrangement of the MYC gene at chromosome 8q24 region, IGH/MYC fusion, and additional signal for IGH gene. We present herein a case of sporadic Burkitt lymphoma occurring in a previously healthy HIV-negative male. The unusual clinical findings in this case include the relatively older age at presentation (55 years, an immunocompetent patient who had nodal involvement and leukemic phase of Burkitt, coupled with partial deafness. A brief educational review of this neoplasm is made.

  6. Interleukin-12 in Treating Patients With Previously Treated Non-Hodgkin's Lymphoma or Hodgkin's Disease

    Science.gov (United States)

    2015-04-14

    Extranodal Marginal Zone B-cell Lymphoma of Mucosa-associated Lymphoid Tissue; Nodal Marginal Zone B-cell Lymphoma; Recurrent Adult Diffuse Large Cell Lymphoma; Recurrent Adult Diffuse Mixed Cell Lymphoma; Recurrent Adult Diffuse Small Cleaved Cell Lymphoma; Recurrent Adult Hodgkin Lymphoma; Recurrent Adult Immunoblastic Large Cell Lymphoma; Recurrent Cutaneous T-cell Non-Hodgkin Lymphoma; Recurrent Grade 1 Follicular Lymphoma; Recurrent Grade 2 Follicular Lymphoma; Recurrent Grade 3 Follicular Lymphoma; Recurrent Mantle Cell Lymphoma; Recurrent Marginal Zone Lymphoma; Recurrent Mycosis Fungoides/Sezary Syndrome; Recurrent Small Lymphocytic Lymphoma; Splenic Marginal Zone Lymphoma; Waldenström Macroglobulinemia

  7. Primary diffuse large B-cell lymphoma of the corpora cavernosa presented as a perineal mass

    Science.gov (United States)

    Carlos, González-Satué; Ivanna, Valverde Vilamala; Gustavo, Tapia Melendo; Joan, Areal Calama; Javier, Sanchez Macias; Luis, Ibarz Servio

    2012-01-01

    Primary male genital lymphomas may appear rarely in testis, and exceptionally in the penis and prostate, but there is not previous evidence of a lymphoma arising from the corpora cavernosa. We report the first case in the literature of a primary diffuse cell B lymphoma of the corpora cavernosa presented with low urinary tract symptoms, perineal pain and palpable mass. Diagnosis was based on trucut biopsy, histopathological studies and computed tomographic images. PMID:22919138

  8. Disseminated intravascular large-cell lymphoma with initial presentation mimicking Guillain-Barré syndrome.

    Science.gov (United States)

    Jiang, Qin Li; Pytel, Peter; Rowin, Julie

    2010-07-01

    We report a patient with intravascular large B-cell lymphoma who initially presented with acute ascending weakness and sensory changes. Electrodiagnostic testing and cerebral spinal fluid (CSF) studies were initially suggestive of a demyelinating polyneuropathy. Further clinical evaluation and testing were consistent with mononeuropathy multiplex. Autopsy revealed disseminated intravascular large-cell lymphoma. Intravascular large-cell lymphoma should be considered in the differential diagnosis of a rapidly evolving neuropathy associated with other organ involvement.

  9. Disease characteristics, treatment patterns, and outcomes of follicular lymphoma in patients 40 years of age and younger: an analysis from the National Lymphocare Study†

    Science.gov (United States)

    Casulo, C.; Day, B.; Dawson, K. L.; Zhou, X.; Flowers, C. R.; Farber, C. M.; Hainsworth, J. D.; Cerhan, J. R.; Link, B. K.; Zelenetz, A. D.; Friedberg, J. W.

    2015-01-01

    Background Follicular lymphoma (FL) is the most common indolent non-Hodgkin lymphoma, with median age at diagnosis in the seventh decade. FL in young adults (YAs), defined as diagnosis at ≤40 years, is uncommon. No standard approaches exist guiding the treatment of YA FL, and little is known about their disease characteristics and outcomes. To gain further insights into YA FL, we analyzed the National LymphoCare Study (NLCS) to describe characteristics, initial treatments, and outcomes in this population versus patients aged >40 years. Patients and methods Using the NLCS database, we stratified FL patients by age: 18–40 (YA), 41–60, 61–70, 71–80, and >80 years. Survival probability was estimated using Kaplan–Meier methodology. We examined associations between age and survival using hazard ratios and 95% confidence intervals (CIs) from multivariable Cox models. Results Of 2652 eligible FL patients in the NLCS, 164 (6%) were YAs. Of YA patients, 69% had advanced disease, 80% had low-grade histology, and 50% had good-risk disease according to the Follicular Lymphoma International Prognostic Index (FLIPI). Nineteen percent underwent observation, 12% received rituximab monotherapy, and 46% received chemoimmunotherapy [in 59% of these: R-CHOP (rituximab plus cyclophosphamide, doxorubicin, vincristine, and prednisone)]. With a median follow-up of 8 years, overall survival (OS) at 2, 5, and 8 years was 98% (95% CI 93–99), 94% (95% CI 89–97), and 90% (95% CI 83–94), respectively. Median progression-free survival (PFS) was 7.3 years (95% CI 5.6–not reached). Conclusions In one of the largest cohorts of YA FL patients treated in the rituximab era, disease characteristics and outcomes were similar to patients aged 41–60 years, with favorable OS and PFS in YAs. Longer-term outcomes and YA-specific survivorship concerns should be considered when defining management. These data may not support the need for more aggressive therapies in YA FL. Clinical trial

  10. Sporadic Burkitt Lymphoma Presenting as Acute Pancreatitis, Concurrent Sinusitis, and Enlarged Adenoids

    OpenAIRE

    2016-01-01

    Pancreatitis and sinusitis as presentations of Burkitt lymphoma are uncommon and rarely described in children. We describe here the case of a child who presented with symptoms suggestive of sinusitis unresponsive to antibiotics, with subsequent development of abdominal symptoms due to pancreatitis. He was eventually diagnosed with Burkitt lymphoma.

  11. Antecedent presentation of aplastic anemia in a patient with diffuse large B cell lymphoma

    Directory of Open Access Journals (Sweden)

    Chien-Ting Chen

    2016-12-01

    Full Text Available Immunological manifestation occasionally develops concurrently with lymphoid neoplasms, including immune thrombocytopenia and autoimmune hemolytic anemia, but rarely reported acquired aplastic anemia (AA. Here we present a female case of diffuse large B cell lymphoma (DLBCL with antecedent presentation of AA. Recovery of AA was noted after complete response to lymphoma treatment. Literature regarding this issue was reviewed.

  12. Follicular Thyroid Cancer Presenting as a Pelvic Mass: A Case Report

    Directory of Open Access Journals (Sweden)

    Halit Karaca

    2011-03-01

    Full Text Available AbstractDistant metastasis is uncommon in differentiated thyroid cancer (DTC and 7% to 23% of DTC patients develop distant metastasis. The remarkably good prognosis and long-term survival in DTC are significantly reduced in patients with distant metastasis as those at the pelvic site. We report the rare case of a patient wth follicular thyroid cancer initially diagnosed as a pelvic mass. Turk Jem 2011; 15: 23-5

  13. Malignant lymphoma of spleen presenting as acute pancreatitis: A case report

    Institute of Scientific and Technical Information of China (English)

    Chao-Ming Wu; Lung-Chih Cheng; Gin-Ho Lo; Kwok-Hung Lai; Chia-Ling Cheng; Wen-Cheng Pan

    2007-01-01

    This is a case report of a patient who presented with acute pancreatitis without the common causes. A pancreatic biopsy revealed large B cell lymphoma. Spleen lymphoma with pancreatic involvement inducing acute pancreatitis, which is a rare disorder, was diagnosed.Here we also review the few similar cases reported in the literature.

  14. FAU in Treating Patients With Advanced Solid Tumors or Lymphoma

    Science.gov (United States)

    2014-01-06

    Adult Grade III Lymphomatoid Granulomatosis; Adult Nasal Type Extranodal NK/T-cell Lymphoma; Anaplastic Large Cell Lymphoma; Angioimmunoblastic T-cell Lymphoma; Cutaneous B-cell Non-Hodgkin Lymphoma; Extranodal Marginal Zone B-cell Lymphoma of Mucosa-associated Lymphoid Tissue; Nodal Marginal Zone B-cell Lymphoma; Recurrent Adult Burkitt Lymphoma; Recurrent Adult Diffuse Large Cell Lymphoma; Recurrent Adult Diffuse Mixed Cell Lymphoma; Recurrent Adult Diffuse Small Cleaved Cell Lymphoma; Recurrent Adult Grade III Lymphomatoid Granulomatosis; Recurrent Adult Hodgkin Lymphoma; Recurrent Adult Immunoblastic Large Cell Lymphoma; Recurrent Adult Lymphoblastic Lymphoma; Recurrent Adult T-cell Leukemia/Lymphoma; Recurrent Cutaneous T-cell Non-Hodgkin Lymphoma; Recurrent Grade 1 Follicular Lymphoma; Recurrent Grade 2 Follicular Lymphoma; Recurrent Grade 3 Follicular Lymphoma; Recurrent Mantle Cell Lymphoma; Recurrent Marginal Zone Lymphoma; Recurrent Mycosis Fungoides/Sezary Syndrome; Recurrent Small Lymphocytic Lymphoma; Small Intestine Lymphoma; Splenic Marginal Zone Lymphoma; Stage III Adult Burkitt Lymphoma; Stage III Adult Diffuse Large Cell Lymphoma; Stage III Adult Diffuse Mixed Cell Lymphoma; Stage III Adult Diffuse Small Cleaved Cell Lymphoma; Stage III Adult Hodgkin Lymphoma; Stage III Adult Immunoblastic Large Cell Lymphoma; Stage III Adult Lymphoblastic Lymphoma; Stage III Adult T-cell Leukemia/Lymphoma; Stage III Cutaneous T-cell Non-Hodgkin Lymphoma; Stage III Grade 1 Follicular Lymphoma; Stage III Grade 2 Follicular Lymphoma; Stage III Grade 3 Follicular Lymphoma; Stage III Mantle Cell Lymphoma; Stage III Marginal Zone Lymphoma; Stage III Mycosis Fungoides/Sezary Syndrome; Stage III Small Lymphocytic Lymphoma; Stage IV Adult Burkitt Lymphoma; Stage IV Adult Diffuse Large Cell Lymphoma; Stage IV Adult Diffuse Mixed Cell Lymphoma; Stage IV Adult Diffuse Small Cleaved Cell Lymphoma; Stage IV Adult Hodgkin Lymphoma; Stage IV Adult Immunoblastic Large Cell

  15. Primary Non-Hodgkins Lymphoma of the prostate presenting as haematuria

    Science.gov (United States)

    Rizvi, Fahad A; Seshagiri, TV; Antil, Satpal; Koneru, Sheshagiri R

    2011-01-01

    We report a rare case of Primary Non-Hodgkins lymphoma of prostate presenting as an emergency with gross haematuria. A review of literature is also discussed. A 71 year old man presented to Emergency department with gross haematuria and was found to have grossly enlarged right lobe of the prostate on digital rectal examination. Histology confirmed a diffuse large B-cell lymphoma of the prostate. CT scan revealed a para-aortic lymphadenopathy which resolved with chemotherapy followed by radiotherapy. The patient remains disease free more than 5 years after initial diagnosis. The treatment and prognosis of primary lymphoma of prostate is same as with other nodal lymphomas. Primary or secondary lymphoma of the prostate should also be considered in patients presenting with haematuria. Cystoscopy and prostate biopsies should be taken to confirm the diagnosis. Treatment with chemo-radiotherapy can provide lasting benefit. PMID:24950539

  16. Therapy-related acute myeloid leukemia with chromosomal abnormalities involving t(9;22(q34;q11 and t(3;21(q26;q22 during chemotherapy for follicular lymphoma

    Directory of Open Access Journals (Sweden)

    Ogasawara T

    2013-06-01

    Full Text Available This report describes a case of a patient who developed therapy-related acute myeloid leukemia five years after initiating chemotherapy for follicular lymphoma. The patient had been treated with multiple chemotherapeutic regimens, including anthracycline and etoposide (VP-16, as well as with radiation therapy for refractory follicular lymphoma over the preceding five years. The patient subsequently developed myelodysplastic syndrome (MDS with karyotypic abnormalities of monosomy 7 and del (q11; q13.3 followed by acute myeloid leukemia (AML with an additional balanced translocation of t(9;22(q34;q11 and t(3;21(q26;q22. Reverse transcription-polymerase chain reaction amplification of the patient’s RNA showed a fusion transcript of minor BCR-ABL but not EVI1-RUNX1 (AML1 genes. Imatinib therapy resulted in regression of AML, but the patient soon became refractory to chemotherapy and died. Therapy-related acute leukemia develops mostly as non-lymphoid leukemia with unbalanced aberrations of monosomy 7 and 5 or balanced aberrations involving 11q23 and 21q22, but Philadelphia chromosome is uncommon. In addition, simultaneous occurrence of both t(9;22(q34;q11 and t(3;21(q26;q22 balanced aberrations in t-MDS/t-AML is a very rare event. The balanced translocations detected in this case suggest another mechanism by which t-AML can develop after chemotherapy and radiation therapy for follicular lymphoma.

  17. Usefulness of {sup 18}F-fluorodeoxyglucose positron emission tomography in follicular lymphoma management; Apport de la tomographie a emission de positons au {sup 18}F-fluorodeoxyglucose dans la prise en charge des lymphomes folliculaires

    Energy Technology Data Exchange (ETDEWEB)

    Le Dortz, L.; Devillers, A.; Prigent, F.; Bahri, H.; Hervouet, T.; Garin, E. [Centre Eugene-Marquis, Service de Medecine Nucleaire, 35 - Rennes (France); Guibert, S. de.; Lamy, T. [CHU de Rennes, Service d' Hematologie, 35 - Rennes (France); Rolland, Y. [Centre Eugene-Marquis, Service de Radiologie, 35 - Rennes (France); Bayat, S. [CHU de Rennes, Dept. d' Information Medicale, 35 - Rennes (France)

    2009-06-15

    Purpose To assess the usefulness of positron emission tomography/computed tomography in staging, prognosis evaluation and re staging of patients with follicular lymphoma. Patients and methods a retrospective study was performed on 45 patients with untreated biopsy-proven follicular lymphoma who underwent F.D.G.-PET/CT and CT before and after chemo-immunotherapy induction treatment (rituximab combined with cyclophosphamide, doxorubicin, vincristine and prednisone). Results PET/CT detected more nodal (+51%) and extra nodal (+89%) lesions than CT. PET/CT changed Ann Arbor stage in eight patients (18%). Five patients (11%) initially considered with early stage (I/II) were finally managed as advanced stage (III/IV). In this study, initial PET/CT was significantly more accurate to identify patients with poor prognosis than F.L.I.P.I.. Poor prognosis was defined as incomplete therapeutic response or early relapse. Accuracy of PET/CT for therapeutic response assessment was significantly higher than that of CT (0.97 vs 0.64), especially because of its ability to identify inactive residual masses. Beside, post-treatment PET/CT was able to predict patients outcome. The median progression free survival (P.F.S.) was 48 months in the PET/CT negative group as compared to 17.2 months for the group with residual uptake (P < 10-4). Conclusion F.D.G.-PET/CT is a very useful tool for staging, assessing prognosis and therapeutic response of patients with follicular lymphoma. (authors)

  18. Treatment of Hodgkin lymphoma: the past, present, and future

    DEFF Research Database (Denmark)

    Evens, A.M.; Hutchings, M.; Diehl, V.

    2008-01-01

    Significant advances in the biology and treatment of Hodgkin lymphoma (HL) have been accomplished over the past decades. In a landmark study, DeVita and colleagues showed that half of patients with advanced-stage HL experienced long-term disease-free survival following treatment with a four-drug ...

  19. Expression patterns of nicotinamide phosphoribosyltransferase and nicotinic acid phosphoribosyltransferase in human malignant lymphomas

    DEFF Research Database (Denmark)

    Olesen, Uffe Høgh; Hastrup, Nina; Sehested, Maxwell

    2011-01-01

    lymphomas (diffuse large B-cell lymphoma, follicular B-cell lymphoma, Hodgkin's lymphoma and peripheral T-cell lymphoma). The expression of NAMPT was generally high in the more aggressive malignant lymphomas, with >80% strong expression, whereas the expression in the more indolent follicular lymphoma (FL...

  20. Rituximab and Interleukin-12 in Treating Patients With B-Cell Non-Hodgkin's Lymphoma

    Science.gov (United States)

    2013-08-23

    Extranodal Marginal Zone B-cell Lymphoma of Mucosa-associated Lymphoid Tissue; Nodal Marginal Zone B-cell Lymphoma; Recurrent Grade 1 Follicular Lymphoma; Recurrent Grade 2 Follicular Lymphoma; Recurrent Mantle Cell Lymphoma; Recurrent Small Lymphocytic Lymphoma; Splenic Marginal Zone Lymphoma

  1. Dendritic Cell Therapy, Cryosurgery, and Pembrolizumab in Treating Patients With Non-Hodgkin Lymphoma

    Science.gov (United States)

    2017-01-26

    Aggressive Non-Hodgkin Lymphoma; Indolent Non-Hodgkin Lymphoma; Recurrent Adult Non-Hodgkin Lymphoma; Recurrent Diffuse Large B-Cell Lymphoma; Recurrent Follicular Lymphoma; Recurrent Mantle Cell Lymphoma; Recurrent Marginal Zone Lymphoma; Recurrent Mediastinal (Thymic) Large B-Cell Cell Lymphoma; Recurrent T-Cell Non-Hodgkin Lymphoma; Small Lymphocytic Lymphoma

  2. Transformation of a Cutaneous Follicle Center Lymphoma to a Diffuse Large B-Cell Lymphoma—An Unusual Presentation

    Directory of Open Access Journals (Sweden)

    J. Dias Coelho

    2010-01-01

    Full Text Available Primary cutaneous follicle center lymphoma (PCFCL is characterized by a proliferation of follicle center cells in the skin. A definitive diagnosis is frequently delayed because of difficulties in interpretation of the histopathologic findings. It has an excellent prognosis with a 5-year survival over 95% and its risk of transformation has not been established. We describe a case report of man with a gastric diffuse large B-cell lymphoma (DLBCL referred to our clinic because of nodules in the back that had gradually developed over a period of 10 years. A biopsy performed 3 years before was interpreted as reactive follicular hyperplasia. A new skin biopsy revealed a diffuse large B-cell lymphoma and immunoglobulin heavy chain gene rearrangements from the initial skin biopsy (PCBCL and the DLBCL gastric biopsy were studied by polymerase chain reaction and an identical clonal rearrangement was detected which was highly suggestive of a transformation lymphoma.

  3. Central nervous system lymphoma presenting as trigeminal neuralgia: A diagnostic challenge

    Science.gov (United States)

    Ang, Jensen W. J.; Khanna, Arjun; Walcott, Brian P.; Kahle, Kristopher T.; Eskandar, Emad N.

    2015-01-01

    We describe an atypical man with diffuse large B cell lymphoma localized to the sphenoid wing and adjacent cavernous sinus, initially presenting with isolated ipsilateral facial pain mimicking trigeminal neuralgia due to invasion of Meckel’s cave but subsequently progressing to intra-axial extension and having synchronous features of systemic lymphoma. Primary central nervous system lymphoma is uncommon, accounting for approximately 2% of all primary intra-cranial tumors, but its incidence has been steadily increasing in some groups [1]. It usually arises in periventricular cerebral white matter, reports of lymphoma in extra-axial regions are rare [2]. This man highlights the importance of maintaining lymphoma in the differential diagnosis of tumors of the skull base presenting with trigeminal neuralgia-like symptoms. PMID:25865026

  4. Clinical Features and Outcome in Newly Diagnosed Hodgkin Lymphoma Patients Presenting with PET/CT-Ascertained Focal Skeletal Lesions

    DEFF Research Database (Denmark)

    El-Galaly, Tarec Christoffer; Hutchings, Martin; Juul Mylam, Karen;

    Clinical Features and Outcome in Newly Diagnosed Hodgkin Lymphoma Patients Presenting with PET/CT-Ascertained Focal Skeletal Lesions......Clinical Features and Outcome in Newly Diagnosed Hodgkin Lymphoma Patients Presenting with PET/CT-Ascertained Focal Skeletal Lesions...

  5. Clinical Features and Outcome in Newly Diagnosed Hodgkin Lymphoma Patients Presenting with PET/CT-Ascertained Focal Skeletal Lesions

    DEFF Research Database (Denmark)

    El-Galaly, Tarec Christoffer; Hutchings, Martin; Juul Mylam, Karen

    Clinical Features and Outcome in Newly Diagnosed Hodgkin Lymphoma Patients Presenting with PET/CT-Ascertained Focal Skeletal Lesions......Clinical Features and Outcome in Newly Diagnosed Hodgkin Lymphoma Patients Presenting with PET/CT-Ascertained Focal Skeletal Lesions...

  6. Lymphoma of the Internal Auditory Canal Presenting as Facial Palsy, Vertigo, and Hearing Loss.

    Science.gov (United States)

    Ryou, Namhyung; Ko, Dong-yn; Jun, Hyung Jin; Chae, Sung Won

    2015-12-01

    The combined symptoms of vertigo, hearing loss, and facial palsy indicate the presence of lesions in the internal auditory canal (IAC). Differential diagnoses, such as inner/middle ear infections and IAC neoplasms, can make the definitive diagnosis of IAC lymphomas challenging. Lymphomas can infiltrate the central nervous system at various sites; however, IAC involvement in metastatic lymphomas is very rare. Herein we report the case of a patient with IAC lymphoma presenting with aural fullness of the left ear and intractable otalgia followed by symptoms of facial weakness, hearing loss, and vertigo within 48 h. The uncharacteristic clinical manifestations and concurrent middle ear infection meant that the conclusive diagnosis of IAC lymphoma was delayed.

  7. Paravertebral Burkitt's Lymphoma in a Child: An Unusual Presentation

    Directory of Open Access Journals (Sweden)

    C. Hoyoux

    2012-01-01

    Full Text Available Paravertebral malignant tumors constitute 4.8% of cancer cases in pediatric oncology and are mostly composed of neuroblastoma (46.4% and soft tissue sarcomas (35.7%. We describe the case of a Caucasian 6-year-old boy who was admitted for middle back pain radiated to limbs and progressively increasing weakness of the legs, suggesting a spinal cord disease. The exploration revealed two paravertebral masses extending through the neural foraminae into the epidural space. The association with elevated serum neuron specific enolase suggested at first the diagnosis of neuroblastoma, but the pathological examination revealed a Burkitt's lymphoma. This is a rare location of sporadic Burkitt's lymphoma with neurologic syndrome as first symptoms.

  8. [Predictive value of Hodgkin's lymphoma tumor burden in present].

    Science.gov (United States)

    Kulyova, S A; Karitsky, A P

    2014-01-01

    Today approximately 70% of patients with Hodgkin lymphoma can be cured with the combined-modality therapy. Tumor burden, the importance of which was demonstrated 15 years ago for the first time, is a powerful prognostic factor. Data of literature of representations on predictive value of Hodgkin's lymphoma tumor burden are shown in the article. The difficult immunological relations between tumor cells and reactive ones lead to development of the main symptoms. Nevertheless, the collective sign of tumor burden shows the greatest influence on survival and on probability of resistance, which relative risk can be predicted on this variable and treatment program. Patients with bulky disease need escalated therapy with high-dose chemotherapy. Integration into predictive models of the variable will change an expected contribution of clinical and laboratory parameters in the regression analyses constructed on patients with Hodgkin's lymphoma. Today the role of diagnostic functional methods, in particular a positron emission tomography, for metabolic active measurement is conducted which allows excluding a reactive component.

  9. Systemic Capillary Leak Syndrome as an Initial Presentation of ALK-Negative Anaplastic Large Cell Lymphoma

    Directory of Open Access Journals (Sweden)

    Laura S. Lourdes

    2012-01-01

    Full Text Available Systemic capillary leak syndrome (SCLS is a rare disease characterized by third spacing of plasma into the extravascular compartment, leading to anasarca, hemoconcentration, and hypovolemic shock. It has been rarely associated with lymphomas, and reports usually indicate that it occurs after antineoplastic treatment. We present the case of a patient with ALK-negative anaplastic large cell lymphoma who presented with SCLS as the initial manifestation of her lymphoma. The SCLS resolved with treatment of the malignancy with steroids and chemotherapy.

  10. Follicular lymphoma: in vitro effects of combining lymphokine-activated killer (LAK) cell-induced cytotoxicity and rituximab- and obinutuzumab-dependent cellular cytotoxicity (ADCC) activity.

    Science.gov (United States)

    García-Muñoz, Ricardo; López-Díaz-de-Cerio, Ascensión; Feliu, Jesus; Panizo, Angel; Giraldo, Pilar; Rodríguez-Calvillo, Mercedes; Grande, Carlos; Pena, Esther; Olave, Mayte; Panizo, Carlos; Inogés, Susana

    2016-04-01

    Follicular lymphoma (FL) is a disease of paradoxes-incurable but with a long natural history. We hypothesized that a combination of lymphokine-activated killer (LAK) cells and monoclonal antibodies might provide a robust synergistic treatment and tested this hypothesis in a phase II clinical trial (NCT01329354). In this trial, in addition to R-CHOP, we alternated the administration of only rituximab with rituximab and autologous LAK cells that were expanded ex vivo. Our objective was to determine the in vitro capability of LAK cells generated from FL patients to produce cytotoxicity against tumor cell lines and to determine rituximab- and obinutuzumab-induced cytotoxicity via antibody-dependent cellular cytotoxicity (ADCC) activity. We analyzed the LAK cell-induced cytotoxicity and rituximab (R)- and obinutuzumab (GA101)-induced ADCC activity. We show that LAK cells generated from FL patients induce cytotoxicity against tumor cell lines. R and GA101 enhance cytolysis through ADCC activity of LAK cells. Impaired LAK cell cytotoxicity and ADCC activity were detected in 50 % of patients. Percentage of NK cells in LAK infusions were correlated with the R- and GA101-induced ADCC. Our results indicate that the combination of R or GA101 and LAK cells should be an option as frontline maintenance therapy in patients with FL.

  11. FDG-PET is prognostic and predictive for progression-free survival in relapsed follicular lymphoma: exploratory analysis of the GAUSS study.

    Science.gov (United States)

    Kostakoglu, Lale; Goy, Andre; Martinelli, Giovanni; Caballero, Dolores; Crump, Michael; Gaidano, Gianluca; Baetz, Tara; Buckstein, Rena; Fine, Gregg; Fingerle-Rowson, Guenter; Berge, Claude; Sahin, Deniz; Press, Oliver; Sehn, Laurie

    2017-02-01

    An exploratory analysis of 75 follicular lymphoma patients treated with obinutuzumab or rituximab induction therapy (IT) for 4 weeks in the phase II GAUSS study aimed to determine whether positron emission tomography (PET) results could predict progression-free survival (PFS) and tumor response. The proportion of patients with a PFS event (progression or death) was higher in those who were PET-positive after IT (assessed using Deauville five-point scale criteria; 35/52, 67%) than PET-negative (5/20, 25%); the hazard ratio for progression or death was 0.25 (95%CI: 0.01-0.64; p = 0.0018). A significant association was also found when PET results were assessed using International Harmonization Project and European Organisation for Research and Treatment of Cancer criteria. Change between baseline and end of IT in values of standardized uptake value and other PET parameters were associated with PFS and response. Validation of these results in prospective studies of larger cohorts is warranted.

  12. Molecular Evaluation of t(14;18(bcl-2/IgH Translocation in Follicular Lymphoma at Diagnosis Using Paraffin-Embedded Tissue Sections

    Directory of Open Access Journals (Sweden)

    Nur Selvi

    2012-06-01

    Full Text Available OBJECTIVE: Follicular lymphoma (FL is one of the most common lymphomas, and is characterized by t(14;18 (q32;q21 in more than 80% of patients. The aim of this study was to determine the rate of t(14;18 positivity based on the detection of mbr or mcr in paraffin-embedded tissue samples. METHODS: The study included 32 paraffin-embedded tissue samples collected from 32 consecutive FL patients that were diagnosed and followed-up at our hospital between 1999 and 2006. The MBR breakpoint was identified based on real-time PCR using a LightCycler v.2.0 t(14;18 Quantification Kit (MBR, multiplex PCR, and seminested PCR. To identify the mcr breakpoint, real-time PCR was performed using specific primers and the FastStart DNA Master SYBR Green I Kit. To detect t(14;18 via fluorescence in situ hybridization (FISH nuclei from paraffin-embedded tissue sections were extracted and used together with LSI IgH (immunoglobulin heavy chain (spectrum green/bcl-2 (B-cell leukemia-lymphoma 2 (spectrum orange probes. RESULTS: The DNA and nuclei isolation success rate for B5 formalin-fixed, paraffin-embedded tissue sections (n = 12 was 42% and 33%, respectively, versus 95% and 60%, respectively, for 20 tissue sections fixed in formalin only. In all, 24 paraffin-embedded tissue sections were analyzed and mbr positivity was observed in the DNA of 82.14% via seminested PCR, in 53.57% via multiplex PCR, and in 28.57% via real-time PCR. We did not detect mcr rearrangement in any of the samples. In all, 15 of 16 patients (93.75% whose nuclei were successfully isolated were observed to be t(14;18 positive via the FISH method. CONCLUSION: Semi-nested PCR and FISH facilitated the genetic characterization of FL tumors. As such, FISH and PCR complement each other and are both essential for detecting t(14;18 translocation.

  13. Pembrolizumab Alone or With Idelalisib or Ibrutinib in Treating Patients With Relapsed or Refractory Chronic Lymphocytic Leukemia or Other Low-Grade B-Cell Non-Hodgkin Lymphomas

    Science.gov (United States)

    2016-06-02

    Recurrent Chronic Lymphocytic Leukemia; Recurrent Extranodal Marginal Zone Lymphoma of Mucosa-Associated Lymphoid Tissue; Recurrent Grade 1 Follicular Lymphoma; Recurrent Grade 2 Follicular Lymphoma; Recurrent Grade 3 Follicular Lymphoma; Recurrent Lymphoplasmacytic Lymphoma; Recurrent Marginal Zone Lymphoma; Recurrent Nodal Marginal Zone Lymphoma; Recurrent Small Lymphocytic Lymphoma; Recurrent Splenic Marginal Zone Lymphoma; Refractory Chronic Lymphocytic Leukemia; Refractory Extranodal Marginal Zone Lymphoma of Mucosa-Associated Lymphoid Tissue; Refractory Follicular Lymphoma; Refractory Lymphoplasmacytic Lymphoma; Refractory Nodal Marginal Zone Lymphoma; Refractory Small Lymphocytic Lymphoma; Refractory Splenic Marginal Zone Lymphoma; Richter Syndrome; Waldenstrom Macroglobulinemia

  14. Intravascular large B-cell lymphoma presenting clinically as rapidly progressive dementia.

    Science.gov (United States)

    Brett, F M; Chen, D; Loftus, T; Langan, Y; Looby, S; Hutchinson, S

    2017-07-19

    In patients presenting with rapidly progressive dementia, prion disease may enter the differential diagnosis. The commonest malignancies masquerading as prion disease are primary CNS lymphoma and intravascular large B-cell lymphoma, both rare and difficult to diagnose without brain biopsy. This 82-year-old lady with a past history of hypertension, presented with rapidly progressive cognitive impairment and ataxia. The possibility of sCJD was raised. Brain biopsy was carried out. Western blot for prion protein was negative. Brain biopsy showed intravascular large B-cell lymphoma. She died shortly afterwards. The clinical presentation of intravascular large B-cell lymphoma is diverse. Patients may present as in this case with dementia, seizures, and myoclonus leading to a clinical diagnosis of sCJD. The diagnosis here was made at biopsy but is made at autopsy in over 50% of cases.

  15. t(14;18)阴性滤泡性淋巴瘤的分子遗传学机制及研究进展%Molecular Genetic Pathogenesis and Research Advances in Follicular Lymphoma without (14;18)Translocation

    Institute of Scientific and Technical Information of China (English)

    潘毅

    2011-01-01

    滤泡性淋巴瘤(FL)是生发中心起源的低级别B细胞非霍奇金淋巴瘤.最具特征性的遗传学改变是染色体14q32上的免疫球蛋白重链基因(IgH)和染色体18q21上的Bcl-2基因的平衡转位,形成t(14;18)(q32;q21).此转位使Bcl-2蛋白过度表达.在功能上延长了细胞的生存时间并导致FL的形成.t(14;18)并不是出现于所有FL病例中,大约90%的FL存在t(14;18),其余的FL未能检测到该转位.t(14;18)的存在与否与滤池性淋巴瘤的发生和发展密切相关.%Follicular lymphoma ( FL ) is a low-grade B-cell non-Hodgkin's lymphoma of germinal center origin. The genetic hallmark of FL is the chromosomal translocation between the immunoglobulin heavy-chain gene on chromosome 18 and the Bcl-2 gene on chromosome 14. The resultant t ( 14;18 ) ( q32;q21 ) leads to the constitutive overexpression of Bcl-2 protein. Functionally, this phenomenon results in the prolongation of cell survival and lymphomagenesis. However, the translocation of t ( 14; 18 ) is not present in all FL cases. Approximately 90% of all FLs carry this characteristic alteration; in the remaining cases, t ( 14; 18 ) is not detectable. The presence or absence of t (14; 18) is closely linked to the occurrence and development of FLs.

  16. Competitive Transfer of αCD19-TCRz-CD28 and αCD19-TCRz-CD137 CAR-T Cells for B-cell Leukemia/Lymphoma

    Science.gov (United States)

    2016-08-22

    Hematopoietic/Lymphoid Cancer; Adult Acute Lymphoblastic Leukemia in Remission; B-cell Adult Acute Lymphoblastic Leukemia; B-cell Chronic Lymphocytic Leukemia; Recurrent Adult Diffuse Large Cell Lymphoma; Recurrent Grade 1 Follicular Lymphoma; Recurrent Grade 2 Follicular Lymphoma; Recurrent Grade 3 Follicular Lymphoma; Recurrent Mantle Cell Lymphoma; Refractory Chronic Lymphocytic Leukemia; Stage III Adult Diffuse Large Cell Lymphoma; Stage III Chronic Lymphocytic Leukemia; Stage III Grade 1 Follicular Lymphoma; Stage III Grade 2 Follicular Lymphoma; Stage III Grade 3 Follicular Lymphoma; Stage III Mantle Cell Lymphoma; Stage IV Adult Diffuse Large Cell Lymphoma; Stage IV Chronic Lymphocytic Leukemia; Stage IV Grade 1 Follicular Lymphoma; Stage IV Grade 2 Follicular Lymphoma; Stage IV Grade 3 Follicular Lymphoma; Stage IV Mantle Cell Lymphoma

  17. Rare clinical presentation of diffuse large B-cell lymphoma as otitis media and facial palsy.

    Science.gov (United States)

    Siddiahgari, Sirisha Rani; Yerukula, Pallavi; Lingappa, Lokesh; Moodahadu, Latha S

    2016-01-01

    Extra nodal presentation of Non Hodgkins Lymphoma (NHL) is a rare entity, and data available about the NHL that primarily involves of middle ear and mastoid is limited. We report a case of diffuse large B cell lymphoma (DLBCL), in a 2 year 8 month old boy, who developed otalgia and facial palsy. Computed tomography revealed a mass in the left mastoid. Mastoid exploration and histopathological examination revealed DLBCL. This case highlights the importance of considering malignant lymphoma as one of the differential diagnosis in persistent otitis media and/facial palsy.

  18. Primary central nervous system lymphoma presenting as isolated oculomotor nerve palsy

    Directory of Open Access Journals (Sweden)

    Terence Tan, MBBS

    2014-09-01

    Full Text Available The authors report an unusual case of primary central nervous system lymphoma presenting with isolated pupil-involved oculomotor nerve palsy. Magnetic resonance imaging demonstrated leptomeningeal involvement of the midbrain and interpeduncular cistern, a single hypothalamic lesion, and intraventricular involvement. Diffuse large B-cell lymphoma was confirmed by stereotactic intraventricular biopsy. Combination chemotherapy with methotrexate, vincristine, procarbazine and rituximab was instituted with resolution of oculomotor nerve palsy and complete disease remission. An interdisciplinary approach involving neurosurgeons, neuroradiologists, neuropathologists and neurologists is crucial in the management of primary central nervous system lymphoma.

  19. Adult T-cell leukemia/lymphoma presenting multiple lymphomatous polyposis

    Institute of Scientific and Technical Information of China (English)

    Akira Hokama; Nobuyuki Takasu; Jiro Fujita; Takeaki Tomoyose; Yu-ichi Yamamoto; Takako Watanabe; Tetsuo Hirata; Fukunori Kinjo; Seiya Kato; Koichi Ohshima; Hiroshi Uezato

    2008-01-01

    Multiple lymphomatous polyposis (HLP) is an unusual form of non-Hodgkin's lymphoma characterized by polyps throughout the gastrointestinal tract. It has been reported that most MLP are observed in cases with mantle cell lymphoma of B-cell type. We herein present a case of a 66-year-old man with adult T-cell leukemia/lymphoma (ATLL). Colonoscopy revealed MLP throughout the colon and histopathological findings of ATLL cell infiltration. The patient died despite combination of chemotherapy. The literature of manifestations of colonic involvement of ATLL is reviewed and the importance of endoscopic evaluation to differentiate ATLL intestinal lesions from opportunistic infectious enterocolitis is discussed.

  20. An Unusual Presentation of B-Cell Lymphoma as a Large Isolated Epiglottic Mass: Case Report and Literature Review

    Directory of Open Access Journals (Sweden)

    Changxing Liu

    2016-01-01

    Full Text Available Extranodal presentation of B-cell lymphoma is uncommon. Isolated primary epiglottic B-cell lymphoma is even rarer. To our knowledge, there has been only one description of isolated B-cell lymphoma presenting as a large epiglottic mass. We report an unusual type of B-cell lymphoma of the epiglottis, as it could not be subtyped based on routine staining and hybridization. The lymphoma presented as a large isolated globular mass pedicled to the epiglottis, occupying most of the oropharynx, but did not have any ball-valving effect or increased respiratory efforts. Initial radiographic findings were nonspecific. The diagnosis of B-cell lymphoma was determined by transoral incisional biopsy under local anesthesia. The condition was treated successfully with chemoradiation. The current standard of treatment for high grade B-cell lymphoma is concurrent chemoradiotherapy, with excellent prognosis. Although rare, B-cell lymphoma should be considered when investigating pedunculated hypopharyngeal masses.

  1. [Molecular abnormalities in lymphomas].

    Science.gov (United States)

    Delsol, G

    2010-11-01

    Numerous molecular abnormalities have been described in lymphomas. They are of diagnostic and prognostic value and are taken into account for the WHO classification of these tumors. They also shed some light on the underlying molecular mechanisms involved in lymphomas. Overall, four types of molecular abnormalities are involved: mutations, translocations, amplifications and deletions of tumor suppressor genes. Several techniques are available to detect these molecular anomalies: conventional cytogenetic analysis, multicolor FISH, CGH array or gene expression profiling using DNA microarrays. In some lymphomas, genetic abnormalities are responsible for the expression of an abnormal protein (e.g. tyrosine-kinase, transcription factor) detectable by immunohistochemistry. In the present review, molecular abnormalities observed in the most frequent B, T or NK cell lymphomas are discussed. In the broad spectrum of diffuse large B-cell lymphomas microarray analysis shows mostly two subgroups of tumors, one with gene expression signature corresponding to germinal center B-cell-like (GCB: CD10+, BCL6 [B-Cell Lymphoma 6]+, centerine+, MUM1-) and a subgroup expressing an activated B-cell-like signature (ABC: CD10-, BCL6-, centerine-, MUM1+). Among other B-cell lymphomas with well characterized molecular abnormalies are follicular lymphoma (BCL2 deregulation), MALT lymphoma (Mucosa Associated Lymphoid Tissue) [API2-MALT1 (mucosa-associated-lymphoid-tissue-lymphoma-translocation-gene1) fusion protein or deregulation BCL10, MALT1, FOXP1. MALT1 transcription factors], mantle cell lymphoma (cycline D1 [CCND1] overexpression) and Burkitt lymphoma (c-Myc expression). Except for ALK (anaplastic lymphoma kinase)-positive anaplastic large cell lymphoma, well characterized molecular anomalies are rare in lymphomas developed from T or NK cells. Peripheral T cell lymphomas not otherwise specified are a heterogeneous group of tumors with frequent but not recurrent molecular abnormalities

  2. Pancoast syndrome: A rare presentation of non-Hodgkin′s lymphoma

    Directory of Open Access Journals (Sweden)

    Anirban Sarkar

    2013-01-01

    Full Text Available Pancoast syndrome is a common presentation of bronchogenic carcinoma, but other malignancies are rarely cited as its cause. Pancoast syndrome due to non-Hodgkin′s lymphoma is rarely described in the literature. Here, we report a case of Pancoast syndrome due to non-Hodgkin′s lymphoma to increase the awareness of the clinicians regarding essentiality of tissue diagnosis of Pancoast tumor before starting the treatment.

  3. [Muscle tissue lymphoma presenting only with fever of unknown origin: a case report and literature review].

    Science.gov (United States)

    Shen, Xiaodong; Chen, Xin; Xiao, Hongju; Liu, Gang; Zhai, Yongzhi; Xu, Baixuan; Shi, Huaiyin; Li, Tanshi; Zhu, Haiyan

    2015-06-01

    Extra-nodal malignant lymphoma is often characterized by a lack of typical symptoms and positive results of auxiliary examinations, which make diagnosis difficult. In some cases, fever can be the only clinical manifestation. For the lymphoma patients presenting with persistent fever with a duration over 3 weeks, characteristics of fever including time of fever attack, fever type and effects of drugs may have significant value in the diagnosis, especially in the early stage of the disease or in rare cases.

  4. Atypical presentation of Non-Hodgkin Lymphoma (NHL): a case report

    OpenAIRE

    Fabiola Mastropietro; Alessandra Piccini; Giulia Lucignani; Alfonso Rubino; Giancarlo Fiermonte

    2014-01-01

    Lymphomas infrequently cause peripheral nerve complications. These syndromes mostly occur by direct compression or infiltration of nerves (neurolymphomatosis), but may also be due to a remote effect as paraneoplastic syndromes, neurotoxic complications of chemotherapy, antibody-mediated or autoimmune mechanisms.We report the case of a 60-year-old woman who presented with a complex peripheral nervous system involvement as initial manifestation of Non-Hodgkin Lymphoma (NHL). This case sheds lig...

  5. A case of primary pulmonary NK/T cell lymphoma presenting as pneumonia.

    Science.gov (United States)

    Lee, Sangho; Shin, Bongkyung; Yoon, Hyungseok; Lee, Jung Yeon; Chon, Gyu Rak

    2016-01-01

    Primary pulmonary lymphoma, particularly non-B cell lymphomas involving lung parenchyma, is very rare. A 46-year-old male was admitted to the hospital with fever and cough. Chest X-ray showed left lower lobe consolidation, which was considered pneumonia. However, because the patient showed no response to empirical antibiotic therapy, bronchoscopic biopsy was performed for proper diagnosis. The biopsied specimen showed infiltrated atypical lymphocytes with angiocentric appearance. On immunohistochemical staining, these atypical cells were positive for CD3, CD30, CD56, MUM-1, and granzyme B, and labeled for Epstein-Barr virus encoded RNA in situ hybridization. These findings were consistent with NK/T cell lymphoma. We report on a case of primary pulmonary NK/T cell lymphoma presenting as pneumonic symptoms and review the literature on the subject.

  6. Cellular Immunotherapy Following Chemotherapy in Treating Patients With Recurrent Non-Hodgkin Lymphomas, Chronic Lymphocytic Leukemia or B-Cell Prolymphocytic Leukemia

    Science.gov (United States)

    2016-07-29

    Post-transplant Lymphoproliferative Disorder; B-Cell Prolymphocytic Leukemia; Recurrent Adult Burkitt Lymphoma; Recurrent Adult Diffuse Large Cell Lymphoma; Recurrent Grade 1 Follicular Lymphoma; Recurrent Grade 2 Follicular Lymphoma; Recurrent Grade 3 Follicular Lymphoma; Recurrent Mantle Cell Lymphoma; Recurrent Marginal Zone Lymphoma; Recurrent Small Lymphocytic Lymphoma; Refractory Chronic Lymphocytic Leukemia; Refractory Hairy Cell Leukemia; B-Cell Lymphoma, Unclassifiable, With Features Intermediate Between Diffuse Large B-Cell Lymphoma and Burkitt Lymphoma; B-Cell Lymphoma, Unclassifiable, With Features Intermediate Between Diffuse Large B-Cell Lymphoma and Classical Hodgkin Lymphoma; Recurrent Lymphoplasmacytic Lymphoma

  7. Flavopiridol in Treating Patients With Relapsed or Refractory Lymphoma or Multiple Myeloma

    Science.gov (United States)

    2016-06-27

    Adult Lymphocyte Depletion Hodgkin Lymphoma; Adult Lymphocyte Predominant Hodgkin Lymphoma; Adult Mixed Cellularity Hodgkin Lymphoma; Adult Nodular Sclerosis Hodgkin Lymphoma; Anaplastic Large Cell Lymphoma; Angioimmunoblastic T-cell Lymphoma; Extranodal Marginal Zone B-cell Lymphoma of Mucosa-associated Lymphoid Tissue; Nodal Marginal Zone B-cell Lymphoma; Recurrent Adult Diffuse Large Cell Lymphoma; Recurrent Adult Diffuse Mixed Cell Lymphoma; Recurrent Adult Diffuse Small Cleaved Cell Lymphoma; Recurrent Adult Grade III Lymphomatoid Granulomatosis; Recurrent Adult Hodgkin Lymphoma; Recurrent Adult T-cell Leukemia/Lymphoma; Recurrent Cutaneous T-cell Non-Hodgkin Lymphoma; Recurrent Grade 1 Follicular Lymphoma; Recurrent Grade 2 Follicular Lymphoma; Recurrent Grade 3 Follicular Lymphoma; Recurrent Mantle Cell Lymphoma; Recurrent Marginal Zone Lymphoma; Recurrent Mycosis Fungoides/Sezary Syndrome; Recurrent Small Lymphocytic Lymphoma; Refractory Multiple Myeloma; Splenic Marginal Zone Lymphoma; Stage I Multiple Myeloma; Stage II Multiple Myeloma; Stage III Multiple Myeloma; Waldenström Macroglobulinemia

  8. Circulating follicular helper T cells presented distinctively different responses toward bacterial antigens in primary biliary cholangitis.

    Science.gov (United States)

    Zhou, Zun-Qiang; Tong, Da-Nian; Guan, Jiao; Li, Mei-Fang; Feng, Qi-Ming; Zhou, Min-Jie; Zhang, Zheng-Yun

    2017-10-01

    Primary biliary cholangitis (PBC) is a chronic and progressive cholestatic liver disease with unknown causes. The initiation of PBC is associated with bacterial infections and abnormal immune correlates, such as the presence of self-reactive anti-mitochondrial antibodies and shifted balance of T cell subsets. In particular, the CD4(+)CXCR5(+) follicular helper T (Tfh) cells are highly activated in PBC patients and are significantly associated with PBC severity, but the underlying reasons are unknown. In this study, we found that the circulating CD4(+)CXCR5(+) T cells were enriched with the interferon (IFN)-γ-secreting Th1-subtype and the interleukin (IL)-17-secreting Th17-subtype, but not the IL-4-secreting Th2 subtype. We further demonstrated that a host of microbial motifs, including Pam3CSK4, poly(I:C), LPS, imiquimod, and CpG, could significantly stimulate IFN-γ, IL-17, and/or IL-21 from circulating CD4(+)CXCR5(+) T cells in PBC patients, especially in the presence of monocytes and B cells. Whole bacterial cells of Escherichia coli, Novosphingobium aromaticivorans, and Mycobacterium gordonae, could also potently stimulate IFN-γ, IL-17, and/or IL-21 production from circulating CD4(+)CXCR5(+) T cells. But interestingly, while the whole cell could potently stimulate circulating CD4(+)CXCR5(+) T cells from both healthy controls and PBC patients, the cell protein lysate could only potently stimulate circulating CD4(+)CXCR5(+) T cells from PBC patients, but not those from healthy controls, suggesting that circulating CD4(+)CXCR5(+) T cells in PBC patients had distinctive antigen-specificity from those in healthy individuals. Together, these data demonstrated that bacterial antigen stimulation is a potential source of aberrant Tfh cell activation in PBC patients. Copyright © 2017. Published by Elsevier B.V.

  9. Comparative analysis between RQ-PCR and digital droplet PCR of BCL2/IGH gene rearrangement in the peripheral blood and bone marrow of early stage follicular lymphoma.

    Science.gov (United States)

    Cavalli, Marzia; De Novi, Lucia Anna; Della Starza, Irene; Cappelli, Luca Vincenzo; Nunes, Vittorio; Pulsoni, Alessandro; Del Giudice, Ilaria; Guarini, Anna; Foà, Robin

    2017-05-01

    BCL2/IGH rearrangements were analysed by polymerase chain reaction (PCR) at diagnosis in paired peripheral blood (PB) and bone marrow (BM) samples from 67 patients with stage I/II follicular lymphoma (FL). Real time quantitative PCR (RQ-PCR) and digital droplet PCR (ddPCR) were performed in cases with a major breakpoint region (MBR+) at diagnosis and after localized radiotherapy and rituximab administration in order to investigate the applicability of ddPCR. The overall ddPCR/RQ-PCR concordance was 81·9% (113/138 samples) and 97·5% in the 40/138 with quantifiable disease (RQ-PCR≥10(-5) ). At baseline, ddPCR allowed the recovery of a MBR+ marker in 8/18 (44·4%) samples that resulted MBR-negative/minor cluster region-negative/minor BCL2-negative by qualitative PCR. Moreover, the tumour burden at diagnosis significantly predicted progression-free survival (PSF) only when quantified by ddPCR. Paired PB and BM samples analysis demonstrated a high concordance in the detection of BCL2/IGH+ cells by qualitative and quantitative methods; in particular, 40/62 samples were positive by ddPCR (25 PB+/BM+; 9 PB+/BM-; 6 PB-/BM+), with 34/40 (85%) identified by the study of PB only. In conclusion, in localized FL, ddPCR is a promising tool for monitoring minimal residual disease (MRD) that is at least comparable to RQ-PCR and potentially more accurate. PB is a suitable source for serial BCL2/IGH MRD assessments, regardless of the methodology utilized. © 2017 John Wiley & Sons Ltd.

  10. Long-term follow-up of rituximab plus first-line mitoxantrone, chlorambucil, prednisolone and interferon-alpha as maintenance therapy in follicular lymphoma.

    Science.gov (United States)

    Herold, Michael; Scholz, Christian W; Rothmann, Frank; Hirt, Carsten; Lakner, Volker; Naumann, Ralph

    2015-09-01

    The randomised, controlled OSHO#39 study showed promising results using first-line mitoxantrone, chlorambucil and prednisolone (MCP) chemotherapy plus rituximab in patients with advanced symptomatic follicular lymphoma (FL) in need of therapy. The aim of this long-term follow-up was to investigate whether clinical benefits are maintained after up to 9 years of observation. Following the 4-year follow-up of OSHO#39, 77 FL patients who received rituximab plus MCP (R-MCP) and 52 patients who received MCP (129 patients alive and not previously censored in total) were followed for 5 additional years in this prospective, non-interventional, observational study. For the efficacy analysis, data were jointly analysed with OSHO#39 data (FL intention-to-treat population: 105 patients R-MCP, 96 MCP). Patients not included in the 5-year follow-up were censored. For surviving patients, median follow-up was 102 months (R-MCP) and 87 months (MCP). Although median overall survival (OS) was not yet reached, OS was longer for patients with R-MCP compared with MCP (p = 0.0057), with 8-year-survival rates of 76.1 versus 55.9%. Further time-to-event data were substantially longer for the R-MCP group than for MCP alone: median progression-free survival (PFS) was 93.4 versus 34.9 months, and median event-free survival (EFS) 89.6 versus 26.5 months. Unplanned subanalyses of patients with and without interferon maintenance showed improved PFS and EFS without an impact on OS. The addition of rituximab to first-line MCP chemotherapy improves clinical outcomes in advanced FL patients and translates into long-term OS benefits. R-MCP remains a promising standard option for this patient group.

  11. MicroRNA profiles of t(14;18)-negative follicular lymphoma support a late germinal center B-cell phenotype.

    Science.gov (United States)

    Leich, Ellen; Zamo, Alberto; Horn, Heike; Haralambieva, Eugenia; Puppe, Bernhard; Gascoyne, Randy D; Chan, Wing-Chung; Braziel, Rita M; Rimsza, Lisa M; Weisenburger, Dennis D; Delabie, Jan; Jaffe, Elaine S; Fitzgibbon, Jude; Staudt, Louis M; Mueller-Hermelink, Hans-Konrad; Calaminici, Mariarita; Campo, Elias; Ott, German; Hernández, Luis; Rosenwald, Andreas

    2011-11-17

    A total of 90% of follicular lymphomas (FLs) harbor the translocation t(14;18) leading to deregulated BCL2 expression. Conversely, 10% of FLs lack the t(14;18), and the majority of these FLs do not express BCL2. The molecular features of t(14;18)-negative FLs remain largely unknown. We performed microRNA expression analysis in 32 FL grades 1 to 3A, including 17 t(14;18)-positive FLs, 9 t(14;18)-negative FLs without BCL2 expression, and 6 t(14;18)-negative FLs with BCL2 expression. MicroRNA profiles were correlated with corresponding mRNA expression patterns, and potential targets were investigated by quantitative PCR and immunohistochemistry in an independent validation series of 83 FLs. Statistical analysis identified 17 microRNAs that were differentially expressed between t(14;18)-positive FLs and t(14;18)-negative FLs. The down-regulation of miR-16, miR-26a, miR-101, miR-29c, and miR138 in the t(14;18)-negative FL subset was associated with profound mRNA expression changes of potential target genes involving cell cycle control, apoptosis, and B-cell differentiation. miR-16 target CHEK1 showed increased expression in t(14;18)-negative FLs, whereas TCL1A expression was reduced, in line with a partial loss of the germinal center B-cell phenotype in this FL subset. In conclusion, t(14;18)-negative FL have distinct microRNA profiles that are associated with an increased proliferative capacity and a "late" germinal center B-cell phenotype.

  12. MicroRNA profiles of t(14;18)–negative follicular lymphoma support a late germinal center B-cell phenotype

    Science.gov (United States)

    Leich, Ellen; Zamo, Alberto; Horn, Heike; Haralambieva, Eugenia; Puppe, Bernhard; Gascoyne, Randy D.; Chan, Wing-Chung; Braziel, Rita M.; Rimsza, Lisa M.; Weisenburger, Dennis D.; Delabie, Jan; Jaffe, Elaine S.; Fitzgibbon, Jude; Staudt, Louis M.; Mueller-Hermelink, Hans-Konrad; Calaminici, Mariarita; Campo, Elias; Ott, German; Hernández, Luis

    2011-01-01

    A total of 90% of follicular lymphomas (FLs) harbor the translocation t(14;18) leading to deregulated BCL2 expression. Conversely, 10% of FLs lack the t(14;18), and the majority of these FLs do not express BCL2. The molecular features of t(14;18)–negative FLs remain largely unknown. We performed microRNA expression analysis in 32 FL grades 1 to 3A, including 17 t(14;18)–positive FLs, 9 t(14;18)–negative FLs without BCL2 expression, and 6 t(14;18)–negative FLs with BCL2 expression. MicroRNA profiles were correlated with corresponding mRNA expression patterns, and potential targets were investigated by quantitative PCR and immunohistochemistry in an independent validation series of 83 FLs. Statistical analysis identified 17 microRNAs that were differentially expressed between t(14;18)–positive FLs and t(14;18)–negative FLs. The down-regulation of miR-16, miR-26a, miR-101, miR-29c, and miR138 in the t(14;18)-negative FL subset was associated with profound mRNA expression changes of potential target genes involving cell cycle control, apoptosis, and B-cell differentiation. miR-16 target CHEK1 showed increased expression in t(14;18)-negative FLs, whereas TCL1A expression was reduced, in line with a partial loss of the germinal center B-cell phenotype in this FL subset. In conclusion, t(14;18)–negative FL have distinct microRNA profiles that are associated with an increased proliferative capacity and a “late” germinal center B-cell phenotype. PMID:21960592

  13. Rituximab Extended Schedule or Re-Treatment Trial for Low–Tumor Burden Follicular Lymphoma: Eastern Cooperative Oncology Group Protocol E4402

    Science.gov (United States)

    Kahl, Brad S.; Hong, Fangxin; Williams, Michael E.; Gascoyne, Randy D.; Wagner, Lynne I.; Krauss, John C.; Habermann, Thomas M.; Swinnen, Lode J.; Schuster, Stephen J.; Peterson, Christopher G.; Sborov, Mark D.; Martin, S. Eric; Weiss, Matthias; Ehmann, W. Christopher; Horning, Sandra J.

    2014-01-01

    Purpose In low–tumor burden follicular lymphoma (FL), maintenance rituximab (MR) has been shown to improve progression-free survival when compared with observation. It is not known whether MR provides superior long-term disease control compared with re-treatment rituximab (RR) administered on an as-needed basis. E4402 (RESORT) was a randomized clinical trial designed to compare MR against RR. Patients and Methods Eligible patients with previously untreated low–tumor burden FL received four doses of rituximab, and responding patients were randomly assigned to either RR or MR. Patients receiving RR were eligible for re-treatment at each disease progression until treatment failure. Patients assigned to MR received a single dose of rituximab every 3 months until treatment failure. The primary end point was time to treatment failure. Secondary end points included time to first cytotoxic therapy, toxicity, and health-related quality of life (HRQOL). Results A total of 289 patients were randomly assigned to RR or MR. With a median follow-up of 4.5 years, the estimated median time to treatment failure was 3.9 years for patients receiving RR and 4.3 years for those receiving MR (P = .54). Three-year freedom from cytotoxic therapy was 84% for those receiving RR and 95% for those receiving MR (P = .03). The median number of rituximab doses was four patients receiving RR and 18 for those receiving MR. There was no difference in HRQOL. Grade 3 to 4 toxicities were infrequent in both arms. Conclusion In low–tumor burden FL, a re-treatment strategy uses less rituximab while providing disease control comparable to that achieved with a maintenance strategy. PMID:25154829

  14. Expression of Master Regulators of T-cell, Helper T-cell and Follicular Helper T-cell Differentiation in Angioimmunoblastic T-cell Lymphoma.

    Science.gov (United States)

    Matsumoto, Yosuke; Nagoshi, Hisao; Yoshida, Mihoko; Kato, Seiichi; Kuroda, Junya; Shimura, Kazuho; Kaneko, Hiroto; Horiike, Shigeo; Nakamura, Shigeo; Taniwaki, Masafumi

    2017-09-25

    Objective It has been postulated that the normal counterpart of angioimmunoblastic T-cell lymphoma (AITL) is the follicular helper T-cell (TFH). Recent immunological studies have identified several transcription factors responsible for T-cell differentiation. The master regulators associated with T-cell, helper T-cell (Th), and TFH differentiation are reportedly BCL11B, Th-POK, and BCL6, respectively. We explored the postulated normal counterpart of AITL with respect to the expression of the master regulators of T-cell differentiation. Methods We performed an immunohistochemical analysis in 15 AITL patients to determine the expression of the master regulators and several surface markers associated with T-cell differentiation. Results BCL11B was detected in 10 patients (67%), and the surface marker of T-cells (CD3) was detected in all patients. Only 2 patients (13%) expressed the marker of naïve T-cells (CD45RA), but all patients expressed the marker of effector T-cells (CD45RO). Nine patients expressed Th-POK (60%), and 7 (47%) expressed a set of surface antigens of Th (CD4-positive and CD8-negative). In addition, BCL6 and the surface markers of TFH (CXCL13, PD-1, and SAP) were detected in 11 (73%), 8 (53%), 14 (93%), and all patients, respectively. Th-POK-positive/BCL6-negative patients showed a significantly shorter overall survival (OS) than the other patients (median OS: 33.0 months vs. 74.0 months, p=0.020; log-rank test). Conclusion Many of the AITL patients analyzed in this study expressed the master regulators of T-cell differentiation. The clarification of the diagnostic significance and pathophysiology based on the expression of these master regulators in AITL is expected in the future.

  15. The Management of Classical Hodgkin's Lymphoma: Past, Present, and Future

    Directory of Open Access Journals (Sweden)

    S. E. Richardson

    2011-01-01

    Full Text Available The management of classical Hodgkin's lymphoma (CHL is a success story of modern multi-agent haemato-oncology. Prior to the middle of the twentieth century CHL was fatal in the majority of cases. Introduction of single agent radiotherapy (RT demonstrated for the first time that these patients could be cured. Developments in chemotherapy including the mechlorethamine, vincristine, procarbazine and prednisolone (MOPP and Adriamycin, bleomycin, vinblastine and dacarbazine (ABVD regimens have resulted in cure rates of over 80%. Even in relapse, CHL patients can be salvaged with high dose chemotherapy and autologous haematopoietic stem cell transplantation (ASCT. Challenges remain, however, in finding new strategies to manage the small number of patients who continue to relapse or progress. In addition, the young age of many Hodgkin's patients forces difficult decisions in balancing the benefit of early disease control against the survival disadvantage of late toxicity. In this article we aim to summarise past trials, define the current standard of care and appraise future developments in the management of CHL.

  16. Breast Cancer Mimic: Cutaneous B-Cell Lymphoma Presenting as an Isolated Breast Mass

    Directory of Open Access Journals (Sweden)

    Margaret Taghavi

    2014-10-01

    Full Text Available Background: Primary cutaneous B-cell lymphoma typically localizes to the skin, and dissemination to internal organs is rare. Lymphomatous involvement of the breasts is also rare. We describe the clinical and radiological findings of an unusual case of primary cutaneous B-cell lymphoma presenting as an isolated breast mass without associated skin changes. Case Presentation: The patient was a 55-year-old Caucasian female who initially presented with cutaneous B-cell lymphoma around her eyes and forehead with recurrence involving the skin between her breasts. Three years after terminating treatment due to a lack of symptoms, she presented for an annual screening mammogram that found a new mass in her upper inner right breast without imaging signs of cutaneous extension. On physical examination, there were no corresponding skin findings. Due to the suspicious imaging features of the mass that caused concern for primary breast malignancy, she underwent a core biopsy which revealed cutaneous B-cell lymphoma. Conclusion: When evaluating patients with a systemic disease who present with findings atypical for that process, it is important to still consider the systemic disease as a potential etiology, particularly with lymphoma given its reputation as a great mimicker.

  17. Yttrium Y 90 Ibritumomab Tiuxetan, Fludarabine, Radiation Therapy, and Donor Stem Cell Transplant in Treating Patients With Relapsed or Refractory Non-Hodgkin's Lymphoma

    Science.gov (United States)

    2016-03-21

    B-cell Chronic Lymphocytic Leukemia; Nodal Marginal Zone B-cell Lymphoma; Recurrent Adult Burkitt Lymphoma; Recurrent Adult Diffuse Large Cell Lymphoma; Recurrent Adult Diffuse Mixed Cell Lymphoma; Recurrent Adult Diffuse Small Cleaved Cell Lymphoma; Recurrent Adult Grade III Lymphomatoid Granulomatosis; Recurrent Adult Immunoblastic Large Cell Lymphoma; Recurrent Adult Lymphoblastic Lymphoma; Recurrent Grade 1 Follicular Lymphoma; Recurrent Grade 2 Follicular Lymphoma; Recurrent Grade 3 Follicular Lymphoma; Recurrent Mantle Cell Lymphoma; Recurrent Marginal Zone Lymphoma; Recurrent Small Lymphocytic Lymphoma; Splenic Marginal Zone Lymphoma; Waldenström Macroglobulinemia

  18. Bortezomib and Filgrastim in Promoting Stem Cell Mobilization in Patients With Non-Hodgkin Lymphoma or Multiple Myeloma Undergoing Stem Cell Transplant

    Science.gov (United States)

    2016-04-19

    Adult Grade III Lymphomatoid Granulomatosis; B-cell Chronic Lymphocytic Leukemia; Contiguous Stage II Adult Burkitt Lymphoma; Contiguous Stage II Adult Diffuse Large Cell Lymphoma; Contiguous Stage II Adult Diffuse Mixed Cell Lymphoma; Contiguous Stage II Adult Diffuse Small Cleaved Cell Lymphoma; Contiguous Stage II Adult Immunoblastic Large Cell Lymphoma; Contiguous Stage II Adult Lymphoblastic Lymphoma; Contiguous Stage II Grade 1 Follicular Lymphoma; Contiguous Stage II Grade 2 Follicular Lymphoma; Contiguous Stage II Grade 3 Follicular Lymphoma; Contiguous Stage II Mantle Cell Lymphoma; Contiguous Stage II Marginal Zone Lymphoma; Contiguous Stage II Small Lymphocytic Lymphoma; Cutaneous B-cell Non-Hodgkin Lymphoma; Extranodal Marginal Zone B-cell Lymphoma of Mucosa-associated Lymphoid Tissue; Intraocular Lymphoma; Nodal Marginal Zone B-cell Lymphoma; Noncontiguous Stage II Adult Burkitt Lymphoma; Noncontiguous Stage II Adult Diffuse Large Cell Lymphoma; Noncontiguous Stage II Adult Diffuse Mixed Cell Lymphoma; Noncontiguous Stage II Adult Diffuse Small Cleaved Cell Lymphoma; Noncontiguous Stage II Adult Immunoblastic Large Cell Lymphoma; Noncontiguous Stage II Adult Lymphoblastic Lymphoma; Noncontiguous Stage II Grade 1 Follicular Lymphoma; Noncontiguous Stage II Grade 2 Follicular Lymphoma; Noncontiguous Stage II Grade 3 Follicular Lymphoma; Noncontiguous Stage II Mantle Cell Lymphoma; Noncontiguous Stage II Marginal Zone Lymphoma; Noncontiguous Stage II Small Lymphocytic Lymphoma; Progressive Hairy Cell Leukemia, Initial Treatment; Recurrent Adult Burkitt Lymphoma; Recurrent Adult Diffuse Large Cell Lymphoma; Recurrent Adult Diffuse Mixed Cell Lymphoma; Recurrent Adult Diffuse Small Cleaved Cell Lymphoma; Recurrent Adult Grade III Lymphomatoid Granulomatosis; Recurrent Adult Immunoblastic Large Cell Lymphoma; Recurrent Adult Lymphoblastic Lymphoma; Recurrent Grade 1 Follicular Lymphoma; Recurrent Grade 2 Follicular Lymphoma; Recurrent Grade 3 Follicular

  19. RNA-binding protein VICKZ is expressed in a germinal center associated pattern among lymphoma subtypes

    DEFF Research Database (Denmark)

    Natkunam, Y.; Vainer, G.; Zhao, S.C.;

    2005-01-01

    to the cytoplasm. Among 868 non-Hodgkin and Hodgkin lymphomas tested by immunohistochemistry on tissue microarrays, staining for VICKZ protein was present in 76% (126/165) of follicular lymphoma, 78% (155/200) of DLBCL, 90% (9/10) of mediastinal large B-cell lymphoma, and 100% (2/2) of Burkitt lymphoma. A subset...... of mantle cell lymphoma (11%, 2/19), extranodal (8%, 2/25), and nodal (20%, 1/5) marginal zone lymphoma and lymphoblastic lymphoma (25%, 4/13), showed VICKZ staining. The majority of lymphocyte predominant Hodgkin (92%, 12/13) and classical Hodgkin (94%, 101/108) lymphoma were found to be positive. Among T......Recent effort in the molecular characterization of diffuse large B-cell lymphoma (DLBCL) has led to the recognition that patients with DLBCL of germinal center origin exhibit a better overall survival. Thus, identification and characterization of markers of germinal center derivation...

  20. An unusual presentation of gastric mucosa-associated lymphoid tissue (MALT-type lymphoma

    Directory of Open Access Journals (Sweden)

    Bikram Shrestha

    2016-09-01

    Full Text Available Mucosa-associated lymphoid tissue (MALT-type lymphoma is a relatively rare disease; nevertheless, it is the third most common lymphoma type, accounting for 5–7% of all non-Hodgkin lymphomas. Case series and retrospective analysis published in the literature have suggested that extra gastrointestinal (GI MALT-type lymphoma can occur simultaneously with MALT-type lymphoma involving the GI tract. We report the case of a healthy, 64-year-old Caucasian male who presented with progressive fatigue, non-productive cough, and worsening exertional shortness of breath for 3 months who was subsequently diagnosed with gastric extra-nodal marginal zone B-cell lymphoma or MALToma with simultaneous metastasis to the lung (bronchi based on biopsy reports. Case presentation: A 64-year-old Caucasian male presented to the emergency room complaining of progressive fatigue for 3 months which had progressed to the point of hindering his usual activities of daily living (ADL. He had recently visited his primary care provider for evaluation of a non-productive cough and exertional shortness of breath. A chest radiography obtained at the time showed bilateral infiltrates. He was then treated for atypical pneumonia but his symptoms unfortunately did not improve. Initial investigations in the emergency room revealed severe anemia and a positive stool guaiac test. Imaging showed bilateral pulmonary infiltrates and an irregular gastric mass. Gastric and transbronchial biopsies were suggestive of extra-nodal marginal zone B-cell lymphoma with simultaneous metastasis to the bronchi. He was treated symptomatically with transfusion of packed red blood cells (PRBC and intravenous iron followed by radiotherapy. Helicobacter pylori infection was ruled out eliminating the possibility of treating him with eradication therapy. Conclusion: Although the stomach is the most common and most extensively studied site of involvement of MALT lymphomas, they can also emerge in many other

  1. Hodgkin's lymphoma presenting with markedly elevated IgE: a case report

    Directory of Open Access Journals (Sweden)

    Ellis Anne K

    2009-12-01

    Full Text Available Abstract Background Markedly elevated IgE as a manifestation of a lymphoproliferative disorder has been only rarely reported. Case Presentation We present the case of a 22 year old female referred to the adult Allergy & Clinical Immunology clinic for an extremely elevated IgE level, eventually diagnosed with Hodgkin's lymphoma. She had no history of atopy, recurrent infections, eczema or periodontal disease; stool was negative for ova & parasites. Chest X-ray revealed large bilateral anterior mediastinal masses that demonstrated prominent uptake on gallium scan. Mediastinal lymph node biopsy was consistent with Hodgkin's lymphoma, nodular sclerosing subtype, grade I/II. Conclusion Although uncommon, markedly elevated IgE may be a manifestation of a malignant process, most notably both Hodgkin's and Non-Hodgkin's lymphomas. This diagnosis should be considered in evaluating an otherwise unexplained elevation of IgE.

  2. Primary Bilateral Non-Hodgkin’s Lymphoma of the Adrenal Gland Presenting as Incidental Adrenal Masses

    Directory of Open Access Journals (Sweden)

    Christopher Rizzo

    2015-01-01

    Full Text Available Although lymphoma may occasionally involve the adrenal glands as part of a generalized disease process, primary adrenal lymphoma (PAL is a rare disease. We present a case of a 62-year-old woman with a history of mild/moderate hereditary spherocytosis with a well-compensated baseline haemoglobin, who presented with rapidly progressive symptomatic anaemia. During the diagnostic workup, imaging revealed bilateral large adrenal masses and she was later diagnosed with diffuse large B-cell non-Hodgkin’s lymphoma (DLBCL, with the adrenal glands being the dominant site of the disease. The patient was started on systemic chemotherapy, but her disease progressed with neurological involvement which responded to second-line therapy. Her adrenal disease however was refractory to further therapy.

  3. Lymphocyte Rich Hodgkin's Lymphoma Presented with Warm Hemolytic Anemia: A Case Report and Literature Review

    Directory of Open Access Journals (Sweden)

    Jorge M. Hurtado-Cordovi

    2011-01-01

    Full Text Available Hodgkin's lymphoma accounts for ten percent of all lymphomas. In the United States, there are about 8000 new cases every year. This paper describes a case of lymphocyte-rich Hodgkin's lymphoma (LRHL manifested by autoimmune hemolytic anemia (AIHA. A 27-year-old Israeli male presented with dizziness associated with one month of low-grade fevers and night sweats; he also complained of persistent cough, pruritus, and ten-pound weight lost during this time. The CBC revealed hemoglobin of 5.9 gm/dL, and direct Coomb's test detected multiple nonspecific antibodies consistent with the diagnosis of AIHA. Chest, abdomen, and pelvic CT scan showed mediastinal lymphadenopathy and splenomegaly. Lymph node biopsy revealed classic LRHL. AIHA resolved after completion of the first cycle of chemotherapy with adriamycin, bleomycin, vinblastine, and dacarbazine (ABVD; after six cycles, he went into complete remission. Although infrequent, AIHA can be responsible for the presenting symptoms of HL.

  4. Ocular Adnexal Lymphoma Presenting as Refractory Unilateral Open-angle Glaucoma.

    Science.gov (United States)

    Sears, Nathaniel C; Singh, Annapurna; Singh, Arun D

    2016-08-01

    We report a single case of masquerade glaucoma caused by increased episcleral venous pressure from adnexal lymphoma. The patient presented as a referral for unilateral glaucoma with intraocular pressures (IOPs) consistently >40 mm Hg (right eye). We present data conclusively demonstrating extranodal marginal zone lymphoma of mucosa-associated lymphoid tissue in the involved eye, and provide an account of the treatment of the tumor with sustained regression and complete resolution of his elevated IOP. We conclude with a discussion of the proposed mechanism. This case serves as a reminder that unilateral open-angle glaucoma can be a sign of indolent ocular adnexal lymphoma. The case also provides a useful model for increased IOP with orbital lesions.

  5. Ileocecal Burkitt's Lymphoma Presenting as Ileocolic Intussusception With Appendiceal Invagination and Acute Appendicitis

    Directory of Open Access Journals (Sweden)

    Sheng-Mine Wang

    2010-06-01

    Full Text Available Intussusception is a common cause of abdominal pain in children. Although most cases are idiopathic, about 10% of cases have a pathologic lead point. Burkitt's lymphoma is not a common etiology. Burkitt's lymphoma might present primarily as intussusception in children but has rarely been associated with appendicitis. We report a case in which a 10-year-old obese boy who initially presented with acute appendicitis due to ileocolic intussusception with appendiceal invagination. He underwent one-trocar laparoscopy and antibiotic treatment. The symptoms recurred 10 days after discharge. Colonoscopy disclosed ileocecal Burkitt's lymphoma as the pathological lead point. This case emphasizes the importance of the age of the patient and the anatomic location of the intussusception related to possible etiology, and hence the most appropriate surgical procedure.

  6. Intravascular lymphoma presenting as a specific pulmonary embolism and acute respiratory failure: a case report

    Directory of Open Access Journals (Sweden)

    Georgin-Lavialle Sophie

    2009-05-01

    Full Text Available Abstract Introduction The occurrence of an intravascular lymphoma with severe pulmonary involvement mimicking pulmonary embolism is described. Case presentation A 38-year-old man was referred to our intensive care unit with acute respiratory failure and long lasting fever. Appropriate investigations failed to demonstrate any bacterial, viral, parasitic or mycobacterial infection. A chest computed tomography scan ruled out any proximal or sub-segmental pulmonary embolism but the ventilation/perfusion lung scan concluded that there was a high probability of pulmonary embolism. The cutaneous biopsy pathology diagnosed intravascular lymphoma. Conclusion Intravascular lymphoma is a rare disease characterized by exclusive or predominant growth of neoplastic cells within the lumina of small blood vessels. Lung involvement seems to be common, but predominant lung presentation of this disease is rare. In our patient, urgent chemotherapy, along with adequate supportive care allowed complete recovery.

  7. Genetically Modified Peripheral Blood Stem Cell Transplant in Treating Patients With HIV-Associated Non-Hodgkin or Hodgkin Lymphoma

    Science.gov (United States)

    2015-05-06

    Adult Nasal Type Extranodal NK/T-cell Lymphoma; AIDS-related Diffuse Large Cell Lymphoma; AIDS-related Diffuse Mixed Cell Lymphoma; AIDS-related Diffuse Small Cleaved Cell Lymphoma; AIDS-related Immunoblastic Large Cell Lymphoma; AIDS-related Lymphoblastic Lymphoma; AIDS-related Peripheral/Systemic Lymphoma; AIDS-related Small Noncleaved Cell Lymphoma; Anaplastic Large Cell Lymphoma; Angioimmunoblastic T-cell Lymphoma; Cutaneous B-cell Non-Hodgkin Lymphoma; Extranodal Marginal Zone B-cell Lymphoma of Mucosa-associated Lymphoid Tissue; Hepatosplenic T-cell Lymphoma; HIV-associated Hodgkin Lymphoma; Intraocular Lymphoma; Nodal Marginal Zone B-cell Lymphoma; Noncutaneous Extranodal Lymphoma; Peripheral T-cell Lymphoma; Recurrent Adult Burkitt Lymphoma; Recurrent Adult Diffuse Large Cell Lymphoma; Recurrent Adult Diffuse Mixed Cell Lymphoma; Recurrent Adult Diffuse Small Cleaved Cell Lymphoma; Recurrent Adult Grade III Lymphomatoid Granulomatosis; Recurrent Adult Hodgkin Lymphoma; Recurrent Adult Immunoblastic Large Cell Lymphoma; Recurrent Adult Lymphoblastic Lymphoma; Recurrent Adult T-cell Leukemia/Lymphoma; Recurrent Cutaneous T-cell Non-Hodgkin Lymphoma; Recurrent Grade 1 Follicular Lymphoma; Recurrent Grade 2 Follicular Lymphoma; Recurrent Grade 3 Follicular Lymphoma; Recurrent Mantle Cell Lymphoma; Recurrent Marginal Zone Lymphoma; Recurrent Mycosis Fungoides/Sezary Syndrome; Recurrent Small Lymphocytic Lymphoma; Refractory Hairy Cell Leukemia; Small Intestine Lymphoma; Splenic Marginal Zone Lymphoma; Stage I AIDS-related Lymphoma; Stage II AIDS-related Lymphoma; Stage III AIDS-related Lymphoma; Stage IV AIDS-related Lymphoma; T-cell Large Granular Lymphocyte Leukemia; Testicular Lymphoma; Waldenström Macroglobulinemia

  8. Dose Monitoring of Busulfan and Combination Chemotherapy in Hodgkin or Non-Hodgkin Lymphoma Undergoing Stem Cell Transplant

    Science.gov (United States)

    2015-08-12

    Adult Grade III Lymphomatoid Granulomatosis; Adult Nasal Type Extranodal NK/T-cell Lymphoma; Anaplastic Large Cell Lymphoma; Angioimmunoblastic T-cell Lymphoma; Childhood Burkitt Lymphoma; Childhood Diffuse Large Cell Lymphoma; Childhood Grade III Lymphomatoid Granulomatosis; Childhood Immunoblastic Large Cell Lymphoma; Childhood Nasal Type Extranodal NK/T-cell Lymphoma; Contiguous Stage II Adult Burkitt Lymphoma; Contiguous Stage II Adult Diffuse Large Cell Lymphoma; Contiguous Stage II Adult Diffuse Mixed Cell Lymphoma; Contiguous Stage II Adult Diffuse Small Cleaved Cell Lymphoma; Contiguous Stage II Adult Immunoblastic Large Cell Lymphoma; Contiguous Stage II Adult Lymphoblastic Lymphoma; Contiguous Stage II Grade 1 Follicular Lymphoma; Contiguous Stage II Grade 2 Follicular Lymphoma; Contiguous Stage II Grade 3 Follicular Lymphoma; Contiguous Stage II Mantle Cell Lymphoma; Contiguous Stage II Marginal Zone Lymphoma; Contiguous Stage II Small Lymphocytic Lymphoma; Cutaneous B-cell Non-Hodgkin Lymphoma; Extranodal Marginal Zone B-cell Lymphoma of Mucosa-associated Lymphoid Tissue; Hepatosplenic T-cell Lymphoma; Intraocular Lymphoma; Nodal Marginal Zone B-cell Lymphoma; Noncontiguous Stage II Adult Burkitt Lymphoma; Noncontiguous Stage II Adult Diffuse Large Cell Lymphoma; Noncontiguous Stage II Adult Diffuse Mixed Cell Lymphoma; Noncontiguous Stage II Adult Diffuse Small Cleaved Cell Lymphoma; Noncontiguous Stage II Adult Immunoblastic Large Cell Lymphoma; Noncontiguous Stage II Adult Lymphoblastic Lymphoma; Noncontiguous Stage II Grade 1 Follicular Lymphoma; Noncontiguous Stage II Grade 2 Follicular Lymphoma; Noncontiguous Stage II Grade 3 Follicular Lymphoma; Noncontiguous Stage II Mantle Cell Lymphoma; Noncontiguous Stage II Marginal Zone Lymphoma; Noncontiguous Stage II Small Lymphocytic Lymphoma; Noncutaneous Extranodal Lymphoma; Peripheral T-cell Lymphoma; Recurrent Adult Burkitt Lymphoma; Recurrent Adult Diffuse Large Cell Lymphoma; Recurrent Adult

  9. A Rare Presentation of Lymphoma of the Cervix with Cross-Sectional Imaging Correlation

    Directory of Open Access Journals (Sweden)

    Brinda Rao Korivi

    2014-01-01

    Full Text Available Non-Hodgkin’s lymphoma of the cervix is an extremely uncommon entity, with no standard established treatment protocol. A 43-year-old asymptomatic female with a history of dual hit blastic B-cell lymphoma/leukemia in complete remission presented with an incidental cervical mass, which was initially felt to represent a cervical fibroid on computed tomography (CT. It was further evaluated with ultrasound, biopsy, and positron emission tomography-computed tomography (PET-CT, which demonstrated a growing biopsy-proven lymphomatous mass and new humeral head lesion. The patient was started on chemotherapy to control the newly diagnosed humeral head lesion, which then regressed. She then underwent radiation to the cervix with significant improvement in the cervical lymphoma. A review of cross-sectional imaging findings of lymphoma of the cervix is provided, including how to differentiate it from other more common diseases of the cervix. Clinical awareness of rare cervical masses such as lymphoma is very important in order to achieve timely diagnosis and appropriate treatment.

  10. Vorinostat and Decitabine in Treating Patients With Advanced Solid Tumors or Relapsed or Refractory Non-Hodgkin's Lymphoma, Acute Myeloid Leukemia, Acute Lymphocytic Leukemia, or Chronic Myelogenous Leukemia

    Science.gov (United States)

    2014-08-26

    Adult Acute Myeloid Leukemia With 11q23 (MLL) Abnormalities; Adult Acute Myeloid Leukemia With Inv(16)(p13;q22); Adult Acute Myeloid Leukemia With t(15;17)(q22;q12); Adult Acute Myeloid Leukemia With t(16;16)(p13;q22); Adult Acute Myeloid Leukemia With t(8;21)(q22;q22); Blastic Phase Chronic Myelogenous Leukemia; Extranodal Marginal Zone B-cell Lymphoma of Mucosa-associated Lymphoid Tissue; Nodal Marginal Zone B-cell Lymphoma; Recurrent Adult Acute Lymphoblastic Leukemia; Recurrent Adult Acute Myeloid Leukemia; Recurrent Adult Burkitt Lymphoma; Recurrent Adult Diffuse Large Cell Lymphoma; Recurrent Adult Diffuse Mixed Cell Lymphoma; Recurrent Adult Diffuse Small Cleaved Cell Lymphoma; Recurrent Adult Immunoblastic Large Cell Lymphoma; Recurrent Adult Lymphoblastic Lymphoma; Recurrent Grade 1 Follicular Lymphoma; Recurrent Grade 2 Follicular Lymphoma; Recurrent Grade 3 Follicular Lymphoma; Recurrent Mantle Cell Lymphoma; Recurrent Marginal Zone Lymphoma; Recurrent Small Lymphocytic Lymphoma; Secondary Acute Myeloid Leukemia; Splenic Marginal Zone Lymphoma; Stage III Adult Burkitt Lymphoma; Stage III Adult Diffuse Large Cell Lymphoma; Stage III Adult Diffuse Mixed Cell Lymphoma; Stage III Adult Diffuse Small Cleaved Cell Lymphoma; Stage III Adult Immunoblastic Large Cell Lymphoma; Stage III Adult Lymphoblastic Lymphoma; Stage III Grade 1 Follicular Lymphoma; Stage III Grade 2 Follicular Lymphoma; Stage III Grade 3 Follicular Lymphoma; Stage III Mantle Cell Lymphoma; Stage III Marginal Zone Lymphoma; Stage III Small Lymphocytic Lymphoma; Stage IV Adult Burkitt Lymphoma; Stage IV Adult Diffuse Large Cell Lymphoma; Stage IV Adult Diffuse Mixed Cell Lymphoma; Stage IV Adult Diffuse Small Cleaved Cell Lymphoma; Stage IV Adult Immunoblastic Large Cell Lymphoma; Stage IV Adult Lymphoblastic Lymphoma; Stage IV Grade 1 Follicular Lymphoma; Stage IV Grade 2 Follicular Lymphoma; Stage IV Grade 3 Follicular Lymphoma; Stage IV Mantle Cell Lymphoma; Stage IV Marginal Zone Lymphoma

  11. Atypical presentation of Non-Hodgkin Lymphoma (NHL: a case report

    Directory of Open Access Journals (Sweden)

    Fabiola Mastropietro

    2014-12-01

    Full Text Available Lymphomas infrequently cause peripheral nerve complications. These syndromes mostly occur by direct compression or infiltration of nerves (neurolymphomatosis, but may also be due to a remote effect as paraneoplastic syndromes, neurotoxic complications of chemotherapy, antibody-mediated or autoimmune mechanisms.We report the case of a 60-year-old woman who presented with a complex peripheral nervous system involvement as initial manifestation of Non-Hodgkin Lymphoma (NHL. This case sheds light on “protean” mechanism of peripheral nerve complications during the course of NHL and related diagnostic dilemma.http://dx.doi.org/10.7175/cmi.v8i4.942 

  12. Diffuse large B cell lymphoma presenting as a peri-anal abscess.

    Science.gov (United States)

    Jayasekera, Hasanga; Gorissen, Kym; Francis, Leo; Chow, Carina

    2014-06-04

    A non-healing peri-anal abscess can be difficult to manage and is often attributed to chronic disease. This case documents a male in his seventh decade who presented with multiple peri-anal collections. The abscess cavity had caused necrosis of the internal sphincter muscles resulting in faecal incontinence. Biopsies were conclusive for diffuse large B-cell lymphoma. A de-functioning colostomy was performed and the patient was initiated on CHOP-R chemotherapy. Anal lymphoma masquerading as a peri-anal abscess is rare. A high degree of suspicion must be maintained for an anal abscess which does not resolve with conservative management.

  13. Diffuse large B cell lymphoma presenting as a peri-anal abscess

    OpenAIRE

    Jayasekera, Hasanga; Gorissen, Kym; Francis, Leo; Chow, Carina

    2014-01-01

    A non-healing peri-anal abscess can be difficult to manage and is often attributed to chronic disease. This case documents a male in his seventh decade who presented with multiple peri-anal collections. The abscess cavity had caused necrosis of the internal sphincter muscles resulting in faecal incontinence. Biopsies were conclusive for diffuse large B-cell lymphoma. A de-functioning colostomy was performed and the patient was initiated on CHOP-R chemotherapy. Anal lymphoma masquerading as a ...

  14. Chlorambucil-rituximab as first-line therapy in patients affected by follicular non-Hodgkin's lymphoma: a retrospective single-centre study.

    Science.gov (United States)

    Martinelli, Giovanni; Montoro, Juan; Vanazzi, Anna; Andreola, Giovanna; Liptrott, Sarah; Radice, Davide; Negri, Mara; Preda, Lorenzo; Pruneri, Giancarlo; Laszlo, Daniele

    2015-12-01

    Rituximab, a chimeric monoclonal antibody directed against the CD20 antigen, has been shown to be active in newly diagnosed and relapsed patients with follicular lymphoma (FL), both as monotherapy and in combination with chemotherapy. Many studies suggest that the prognosis of patients with FL may improve when it is used in combination with chemotherapy. Despite these advances, the disease remains essentially incurable with standard therapy, and novel approaches to treatment are needed because optimal therapy is not defined. The combination of chlorambucil-rituximab is one of several standard treatment options for FL. Here, we considered data arising from 75 patients with newly diagnosed FL at the European Institute of Oncology treated with the combination of rituximab plus chlorambucil. The aim of this study was to evaluate the efficacy and safety of chlorambucil and rituximab, delivered 6 mg/m(2) /day orally for 6 weeks and 375 mg/m(2) in a standard 4-weekly schedule, respectively. Patients responding to the induction therapy received a prolonged therapy with four additional cycles of chlorambucil plus rituximab. Seventy-one patients (94.6%) completed the treatment; four patients discontinued treatment because of grade 3-4 hematological toxicity. The overall response rate was 97.3% including 74.7% of complete responses. Only two patients had a stable disease at revaluation after treatment. With a median follow-up of 57 months, 72 patients (96%) are alive. Median event-free survival (EFS) and median overall survival (OS) were not reached; 5-year OS rate was 98.4%. The 5-year EFS was 71.3%. By univariate and multivariate analyses, elevated beta-2 microglobulin levels and partial responses to therapy were correlated with worse EFS. These results suggest that the combination of chlorambucil and rituximab is an active and safe regimen in patients with newly diagnosed FL, principally in those with low tumour burden and favourable prognostic factors. Copyright

  15. Cost-Effectiveness Analysis of Bendamustine Plus Rituximab as a First-Line Treatment for Patients with Follicular Lymphoma in Spain.

    Science.gov (United States)

    Sabater, Eliazar; López-Guillermo, Armando; Rueda, Antonio; Salar, Antonio; Oyagüez, Itziar; Collar, Juan Manuel

    2016-08-01

    Follicular lymphoma (FL) is the second most common type of lymphoid cancer in Western Europe. The aim of this study was to evaluate the cost utility of rituximab-bendamustine treatment compared with R-CHOP (rituximab, cyclophosphamide, doxorubicin, vincristine and prednisone) treatment as a first-line therapy for patients with advanced FL in Spain. A Markov model was developed to estimate the cost effectiveness of rituximab-bendamustine compared with R-CHOP as first-line treatment for patients with advanced FL in the Spanish National Health System (NHS). Transitions between health states (progression-free, including induction and maintenance; first relapse; second relapse; and death) were allowed for the patient cohort in 4-week-long cycles. Clinical data for the extrapolation of progression-free survival curves were obtained from randomized trials. Mortality rates and utilities were obtained from the literature. Outcomes were measured as quality-adjusted life-years (QALYs). The total costs (€, 2013) included drug costs (ex-factory prices with mandatory deductions), disease management costs and adverse event-associated costs. Costs and outcomes were discounted at a 3 % annual rate. Probabilistic sensitivity analysis was performed using 10,000 Monte Carlo simulations to assess the model robustness. Treatment and administration costs during the induction phase were higher for rituximab-bendamustine (€17,671) than for R-CHOP (€11,850). At the end of the 25-year period, the rituximab-bendamustine first-line strategy had a total cost of €68,357 compared with €69,528 for R-CHOP. Health benefits were higher for rituximab-bendamustine treatment (10.31 QALYs) than for R-CHOP treatment (9.82 QALYs). In the probabilistic analysis, rituximab-bendamustine was the dominant strategy over treatment with R-CHOP in 53.4 % of the simulations. First-line therapy with rituximab-bendamustine in FL patients was the dominant strategy over treatment with R-CHOP; it showed cost

  16. The Combination of Rituximab and Bendamustine as First-Line Treatment Is Highly Effective in the Eradicating Minimal Residual Disease in Follicular Lymphoma: An Italian Retrospective Study

    Directory of Open Access Journals (Sweden)

    Sara Galimberti

    2017-06-01

    Full Text Available R-Bendamustine is an effective treatment for follicular lymphoma (FL. Previous large trials demonstrated the prognostic role of the molecular minimal residual disease (MRD during the most frequently adopted chemotherapeutic regimens, but there are not yet conclusive data about the effect of combination of rituximab (R and bendamustine in terms of MRD clearance. Thus, the aim of this retrospective study was to assess if and in what extent the combination of rituximab and bendamustine would exert a significant reduction of the molecular disease in 48 previously untreated FL patients. The molecular marker at baseline was found in the 62.5% of cases; no significant differences were observed between patients with or without the molecular marker in respect of the main clinical features. Moreover, the quantization of the baseline molecular tumor burden showed a great variability: the median value was 1.4 × 10−2 copies, ranging from 3 × 10−5 to 4 × 104. The initial molecular tumor burden did not correlate with clinical features and did not impact on the subsequent quality of response. After treatment, 93% of cases became MRD-negative; the median reduction of the BCL2/JH load was 4 logs. The 2-years PFS was 85%; it was significantly longer for patients in complete than for those in partial response (91 vs. 57%; p = 0.002, and for cases with lower FLIPI-2 score (88 vs. 60%; p = 0.004. On the contrary, PFS did not differ between patients with or without the molecular marker at baseline; a molecular tumor burden 15 times higher was observed in the relapsed subgroup in comparison to the relapse-free one, but this difference did not change the PFS length. The 2-years OS was 93.6%; the only variable that significantly impacted on it was the FLIPI-2 score; the presence of the molecular marker at baseline or its behavior after treatment did not impact on survival. This study, even if retrospective and conducted on a small series of patients, would

  17. Non-Classical Hodgkin's Lymphoma Presenting As Cauda Equina Syndrome-a Rare presentation: One Case Report

    Institute of Scientific and Technical Information of China (English)

    Pradipta Guha; Debasis Sarkar; Indranil Thakur; Partha Sardar; Sekhar Mukherjee; Sanjoy Kumar Chatterjee

    2010-01-01

    @@ Introduction Nodular lymphocyte predominant Hodgkin's disease occurs in 5% of all cases of Hodgkin's disease. It occurs more commonly in male. Only 5% of all Hodgkin's lymphoma cases develop spinal cord compression[1] and in only 0.2% cases, spinal cord compression occurs as the initial presentation[2]. So our case is rare both in the form of the unique variety of Hodgkin's disease and also in the form of presentation.

  18. Acute upper arm ischaemia: a rare presentation of non-Hodgkin's lymphoma.

    LENUS (Irish Health Repository)

    Daruwalla, Z J

    2010-12-01

    Digital ischaemia has been sparsely reported in current literature. Its association with lymphomatous conditions has been described in even more exceptional occurrences. We present the first case of upper arm ischaemia associated with non-Hodgkin\\'s lymphoma. A brief literature review of this rare phenomenon is also accompanied with it.

  19. Primary hepatic lymphoma presenting as fulminant hepatic failure with hyperferritinemia: A case report

    Directory of Open Access Journals (Sweden)

    Haider Fyeza S

    2008-08-01

    Full Text Available Abstract Introduction Primary hepatic lymphoma is an unusual form of non-Hodgkin's lymphoma that usually presents with constitutional symptoms, hepatomegaly and signs of cholestatic jaundice. Diffuse hepatic infiltration is uncommon and presentation with acute hepatic failure even more rare. The presence of markedly elevated ferritin levels can complicate the evaluation process and suggest alternative diagnoses. We present the case of a middle-aged woman exhibiting pancytopenia, hyperferritinemia and rapidly deteriorating to develop acute hepatic failure. Her initial clinical picture led to a working diagnosis of adult onset Still's disease with probable hemophagocytic syndrome before her worsening liver function necessitated a percutaneous liver biopsy and establishment of the final diagnosis of primary hepatic lymphoma. Conclusion Primary hepatic lymphoma is an uncommon malignancy and its manifestation as progressive hepatitis or acute fulminant hepatic failure can be difficult to diagnose. The presence of constitutional symptoms, pancytopenia and high ferritin levels can complicate the evaluation process. A liver biopsy early in the course of liver dysfunction may establish the diagnosis without a higher risk of bleeding complications seen once liver failure sets in.

  20. Skeletal Muscle Metastasis as an Initial Presentation of Follicular Thyroid Carcinoma: A Case Report and a Review of the Literature

    Directory of Open Access Journals (Sweden)

    Mutahir A. Tunio

    2013-01-01

    Full Text Available Introduction. Follicular thyroid carcinoma (FTC frequently metastasizes to the lungs and bones. However, metastasis to the skeletal muscles is an extremely rare manifestation of FTC. To date, only seven cases of FTC have been reported in the literature. Skeletal muscle metastases from FTC usually remain asymptomatic or manifest as swelling and are associated with dismal prognosis. Case Presentation. A 45-year-old Saudi woman presented with right buttock swelling since 8 months. Physical examination revealed right gluteal mass of size  cm and right thyroid lobe nodule. The rest of examination was unremarkable. Magnetic resonance imaging (MRI showed  cm lobulated mass arising from the gluteus medius muscle, and tru-cut biopsy confirmed the metastatic papillary carcinoma of thyroid origin. The patient subsequently underwent palliative radiotherapy followed by total thyroidectomy and radioactive iodine ablation. At the time of publication, the patient was alive with partial response in gluteal mass. Conclusion. Skeletal muscles metastases are a rare manifestation of FTC, and searching for the primary focus in a patient with skeletal muscle metastasis, thyroid cancer should be considered as differential diagnosis.

  1. Alisertib in Combination With Vorinostat in Treating Patients With Relapsed or Recurrent Hodgkin Lymphoma, B-Cell Non-Hodgkin Lymphoma, or Peripheral T-Cell Lymphoma

    Science.gov (United States)

    2016-07-12

    Adult B Acute Lymphoblastic Leukemia; Adult T Acute Lymphoblastic Leukemia; Anaplastic Large Cell Lymphoma; Angioimmunoblastic T-Cell Lymphoma; Chronic Lymphocytic Leukemia; Cutaneous B-Cell Non-Hodgkin Lymphoma; Extranodal Marginal Zone Lymphoma of Mucosa-Associated Lymphoid Tissue; Hepatosplenic T-Cell Lymphoma; Intraocular Lymphoma; Lymphomatous Involvement of Non-Cutaneous Extranodal Site; Mature T-Cell and NK-Cell Non-Hodgkin Lymphoma; Nodal Marginal Zone Lymphoma; Recurrent Adult Acute Lymphoblastic Leukemia; Recurrent Adult Burkitt Lymphoma; Recurrent Adult Diffuse Large Cell Lymphoma; Recurrent Adult Diffuse Mixed Cell Lymphoma; Recurrent Adult Diffuse Small Cleaved Cell Lymphoma; Recurrent Adult Grade III Lymphomatoid Granulomatosis; Recurrent Adult Hodgkin Lymphoma; Recurrent Adult Immunoblastic Lymphoma; Recurrent Adult Lymphoblastic Lymphoma; Recurrent Adult T-Cell Leukemia/Lymphoma; Recurrent Cutaneous T-Cell Non-Hodgkin Lymphoma; Recurrent Grade 1 Follicular Lymphoma; Recurrent Grade 2 Follicular Lymphoma; Recurrent Grade 3 Follicular Lymphoma; Recurrent Mantle Cell Lymphoma; Recurrent Marginal Zone Lymphoma; Recurrent Mycosis Fungoides and Sezary Syndrome; Recurrent Small Lymphocytic Lymphoma; Refractory Chronic Lymphocytic Leukemia; Refractory Hairy Cell Leukemia; Small Intestinal Lymphoma; Splenic Marginal Zone Lymphoma; T-Cell Large Granular Lymphocyte Leukemia; Testicular Lymphoma; Waldenstrom Macroglobulinemia

  2. High-grade GI lymphoma presenting with diarrhoea.

    Science.gov (United States)

    Al-Rubaye, Sinan; Henson, Sophie; Mahdi, Ali

    2013-06-05

    A 63-year-old male carpenter presented with diarrhoea after returning from a holiday in Egypt with no other abdominal features. Physical examination showed generalised lymphadenopathy and splenomegaly. Blood tests including inflammatory markers were within normal limits. Microbiological stool sample analysis was unremarkable. Endoscopies of the large bowel showed patchy inflammation from the rectum to the hepatic flexure with multiple tiny polyps. Histology revealed a lymphocytic infiltrate suggestive of multiple lymphomatous polyposis. Some time later, he presented with small bowel obstruction requiring emergency laparotomy and ileostomy. After recovery from surgery he started definite treatment with chemotherapy resulting in complete remission.

  3. Primary Dural Spinal Lymphoma Presentation of a Rare Spinal Tumor Case

    Directory of Open Access Journals (Sweden)

    Dilber Ayçiçek Çeçen

    2015-01-01

    Full Text Available Background. Primary spinal dural lymphomas (PSDL are tumors with characteristic histopathology of a lymphoma, which are completely in the spinal epidural space without any other systemic involvement. Extranodal primary lymphoma involving nervous system prefers thalamus/basal ganglia, periventricular region, cerebellum, eyes, meninges/dura, and cranial nerves or spinal cord. Rare spinal localization with acute spinal cord compression is worth attention. Case Presentation. A 48-year-old male presented with a several-month-long history of upper back pain. Lately, he had numbness and weakness at both lower extremities and was unable to walk for one week. A spinal MRI showed a thoracic lesion with cord compression at T2–T4 levels. The patient underwent surgical decompression, with his final histopathology showing diffuse large B-cell lymphoma. Systemic work-up was negative for nodal disease. Following surgery, he received radiotherapy combined with chemotherapy. He experienced a good outcome after four years. Conclusion. The upper thoracic cord is a rare location for primary spinal lesions/metastases, both of which prefer the lower thoracic and upper lumbar regions. In cases of progressive paraparesis, there should be immediate surgical intervention in the case of denovo disease, followed by combined radiotherapy and chemotherapy procedures.

  4. Langerhans Cell Histiocytosis in an Infant Mimicking a Lymphoma at Presentation

    Science.gov (United States)

    Madasu, Anjan; Noor Rana, Asim; Banat, Saleh; Humad, Hani; Mustafa, Rashid; AlJassmi, Abdulrahman Mohd

    2015-01-01

    Langerhans cell histiocytosis (LCH) is a rare disorder characterized by proliferation and accumulation of clonal dendritic cells with varied clinical presentation and an unpredictable course. We report a 5-month-old infant with LCH who presented with severe respiratory distress, a large mediastinal mass, significant generalized lymphadenopathy, and hepatosplenomegaly. Lymphoma, especially T cell lymphoblastic lymphoma, can present with superior mediastinal syndrome needing urgent empirical therapy without biopsy. However, lack of response prompted a biopsy which confirmed it to be a case of LCH and that leads to appropriate therapy and survival. There have been reports of LCH presenting with isolated mediastinal mass or with generalized lymphadenopathy, but the combined presentation of generalized lymphadenopathy with large mediastinal mass, hepatosplenomegaly, and fever in an infant has rarely been reported. Conclusion. LCH should also be considered in the differential diagnosis of an infant presenting with generalized lymphadenopathy, mediastinal mass, hepatosplenomegaly, and fever. PMID:26587301

  5. Langerhans Cell Histiocytosis in an Infant Mimicking a Lymphoma at Presentation

    Directory of Open Access Journals (Sweden)

    Anjan Madasu

    2015-01-01

    Full Text Available Langerhans cell histiocytosis (LCH is a rare disorder characterized by proliferation and accumulation of clonal dendritic cells with varied clinical presentation and an unpredictable course. We report a 5-month-old infant with LCH who presented with severe respiratory distress, a large mediastinal mass, significant generalized lymphadenopathy, and hepatosplenomegaly. Lymphoma, especially T cell lymphoblastic lymphoma, can present with superior mediastinal syndrome needing urgent empirical therapy without biopsy. However, lack of response prompted a biopsy which confirmed it to be a case of LCH and that leads to appropriate therapy and survival. There have been reports of LCH presenting with isolated mediastinal mass or with generalized lymphadenopathy, but the combined presentation of generalized lymphadenopathy with large mediastinal mass, hepatosplenomegaly, and fever in an infant has rarely been reported. Conclusion. LCH should also be considered in the differential diagnosis of an infant presenting with generalized lymphadenopathy, mediastinal mass, hepatosplenomegaly, and fever.

  6. Follicular penetration and targeting.

    Science.gov (United States)

    Lademann, Jürgen; Otberg, Nina; Jacobi, Ute; Hoffman, Robert M; Blume-Peytavi, Ulrike

    2005-12-01

    In the past, intercellular penetration was assumed to be the most important penetration pathway of topically applied substances. First hints that follicular penetration needs to be taken into consideration were confirmed by recent investigations, presented during the workshop "Follicular Penetration and Targeting" at the 4th Intercontinental Meeting of Hair Research Societies", in Berlin 2004. Hair follicles represent an efficient reservoir for the penetration of topically applied substances with subsequent targeting of distinct cell populations, e.g., nestin-expressing follicular bulge cells. The volume of this reservoir can be determined by differential stripping technology. The follicular penetration processes are significantly influenced by the state of the follicular infundibulum; recent experimental investigations could demonstrate that it is essential to distinguish between open and closed hair follicles. Topically applied substances can only penetrate into open hair follicle. Knowledge of follicular penetration is of high clinical relevance for functional targeting of distinct follicular regions. Human hair follicles show a hair-cycle-dependent variation of the dense neuronal and vascular network. Moreover, during hair follicle cycling with initiation of anagen, newly formed vessels occur. Thus, the potential of nestin-expressing hair follicle stem cells to form neurons and blood vessels was investigated.

  7. Iodine I 131 Tositumomab and Fludarabine Phosphate in Treating Older Patients Who Are Undergoing an Autologous or Syngeneic Stem Cell Transplant for Relapsed or Refractory Non-Hodgkin's Lymphoma

    Science.gov (United States)

    2014-08-04

    Extranodal Marginal Zone B-cell Lymphoma of Mucosa-associated Lymphoid Tissue; Nodal Marginal Zone B-cell Lymphoma; Recurrent Adult Burkitt Lymphoma; Recurrent Adult Diffuse Large Cell Lymphoma; Recurrent Adult Diffuse Mixed Cell Lymphoma; Recurrent Adult Diffuse Small Cleaved Cell Lymphoma; Recurrent Adult Immunoblastic Large Cell Lymphoma; Recurrent Adult Lymphoblastic Lymphoma; Recurrent Grade 1 Follicular Lymphoma; Recurrent Grade 2 Follicular Lymphoma; Recurrent Grade 3 Follicular Lymphoma; Recurrent Mantle Cell Lymphoma; Recurrent Marginal Zone Lymphoma; Splenic Marginal Zone Lymphoma; Waldenström Macroglobulinemia

  8. Bilateral Non-Hodgkin’s Lymphoma of the Temporal Bone: A Rare and Unusual Presentation

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    Sanjay Vaid

    2016-01-01

    Full Text Available Primary lymphoma of the temporal bone is an unusual finding in clinical practice and bilateral affection is even more rare. To the best of our knowledge, there are no reports of bilateral primary temporal bone lymphoma without middle ear involvement in the English medical literature so far. We report, for the first time, a case of primary lymphoma involving both temporal bones which presented with left-sided infranuclear facial palsy. A combination of contrast enhanced magnetic resonance imaging (MRI and high resolution computed tomography (HRCT was used to characterize and to map the extent of the lesion, as well as to identify the exact site of facial nerve affection. An excision biopsy and immunohistochemistry revealed diffuse large B-cell non-Hodgkin’s lymphoma (DLBCL. Whole body fluorodeoxyglucose (FDG positron emission tomography-computed tomography study (PET-CT was performed to stage the disease. The patient was treated with chemotherapy and radiation therapy and is now on regular follow-up. The patient is alive and asymptomatic without disease progression for the last twenty months after initial diagnosis.

  9. 滤泡变型外周T细胞淋巴瘤临床病理学及遗传学分析%Follicular variant of peripheral T-cell lymphoma: a clinicopathologic and genetic study of 2 cases

    Institute of Scientific and Technical Information of China (English)

    詹鹤琴; 朱雄增; 李小秋; 周晓燕

    2011-01-01

    Objective To observe the clinicopathologic and genetic features of follicular variant of peripheral T-cell lymphoma (FV-PTCL), with particular attention to the relationship of this type of lymphoma with angioimmunoblastic T-cell lymphoma (AIIL). Methods The clinical data, hemstoxylin and eosin-stained sections of lymph node biopsies from 2 FV-PTCL cases were reviewed.Immunohistochemical phenotyping and detection of EBV-encoded RNAs (EBER) through in situ hybridization (ISH) were performed. The EnVision two-step method was used for all antibodies except CXCL13 (by using three-step streptavidin immunoperoxidase method). Analysis of clonality and ITK/SYK gene rearrangement was conducted using PCR and RT-PCR assays, respectively. Results Clinically, the two patients presented with superficial lymphadenopathy similarly. Histologically, case 1 showed a follicular/nodular lymphoid proliferation without marked germinal centers. The neoplastic cells comprised mainly medium sized cells with abundant, sometimes clear cytoplasms. Similar histologic findings were seen in case 2 in addition to a concurrent component mimicking typical AITL noticed. Of both cases, the neoplastic cells showed positive reactivity to CD3, CD4, CD10, PD1, and CXCL13. Positive hybridization signals for EBER were only seen in case 2, and double stains demonstrated that those EBV-positive cells were mostly the reactive transformed B-cells. Monoclonal T-cell proliferation was proved by the rearranged TCR gene detection in both cases. Neither of the current cases expressed ITK/SYK fusion transcripts. Conclusion FV-PTCL shows the similar or overlapped morphological and immunophenotypic features to those of AITL,possibly suggesting the presence of a potential relationship between these two types of lymphomas.%目的 观察滤泡变型外周T细胞淋巴瘤的临床病理特征和遗传学改变,探讨其与血管免疫母细胞性T细胞淋巴瘤的关系.方法 对2例滤泡变型外周T细胞淋巴瘤进

  10. A rare case of primary lymphoma of the caecum presenting as intussusception

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    Leena Jayabackthan

    2013-01-01

    Full Text Available Mucosa associated lymphoid tissue (MALT lymphomas are rare neoplasms. They are most common in the stomach followed by small intestine and colon. The symptoms are nonspecific and generally do not present with intussusception. Here we report a rare clinical entity in which a 35-year-old female presented to the emergency with severe abdominal pain which was sudden in onset. History revealed that she had been having vague mild abdominal pain for 2 years. Ultrasonography showed ileocolic intussusception with hypoechoic lesion of 54 × 46 mm seen at the lead point. Emergency laparotomy with the right hemi-colectomy was done. The specimen was sent for histopathological examination which revealed a diagnosis of MALT lymphoma. Awareness of the varied clinical presentation helps in formulating the appropriate therapeutic strategy.

  11. Occult pulmonary mucosa-associated lymphoid tissue lymphoma presenting as catastrophic antiphospholipid antibody syndrome.

    Science.gov (United States)

    Regunath, Hariharan; Shortridge, James; Raza, Shahzad; Nistala, Puja; Huffman, Brandon M; Wang, Michael X; Xiang, Dong

    2013-11-01

    Catastrophic antiphospholipid antibody syndrome (CAPS) is characterized by fulminant thrombosis of the arterial and venous beds of multiple organ systems over a relatively short period of time and with a high mortality rate. Mucosa-associated lymphoid tissue (MALT) lymphoma of the lung has never been reported as a causative or precipitating factor for CAPS in the CAPS registry database. The present study describes a rare case of pulmonary MALT lymphoma of the lung that presented as CAPS. A 19-year-old Hispanic female presented with shortness of breath and abdominal pain. Computed tomography (CT) scans of the chest and abdomen revealed multiple portal vein thromboses and bilateral pulmonary nodules. Within one week of presentation, the patient developed a straight sinus thrombosis and upper extremity deep vein thrombosis, which led to shortness of breath. A biopsy of the lung nodule revealed MALT lymphoma. The present case illustrates a rarely reported pulmonary MALT lymphoma presenting as CAPS in a young female. The patient was successfully treated with 90 mg/m(2) bendamustine on days one and two and rituximab 375 mg/m(2) on day one of each 28-day cycle. Complete remission of the lung nodules was observed following three cycles of treatment, as visualized by positron emission tomography (PET)/CT scan. Fondaparinux was identified as a feasible anticoagulation drug of choice for this case. At seven months post-treatment, the patient continues to be stable with no further evidence of thrombosis and is currently undergoing rituximab maintenance therapy every six months for two years. A repeat lupus anticoagulant antibody assay turned and remained negative during the clinical follow-up period. A prompt diagnosis and early aggressive treatment is potentially curative and may dramatically decrease the mortality risk. Future studies should explore the role of rituximab in the management of CAPS-associated B-cell lymphoid malignancies.

  12. Intravascular Large B-Cell Lymphoma Presenting as Interstitial Lung Disease

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    Elham Vali Khojeini

    2014-01-01

    Full Text Available Intravascular large B-cell lymphoma (IVLBL is a rare subtype of diffuse large B-cell lymphoma that resides in the lumen of blood vessels. Patients typically present with nonspecific findings, particularly bizarre neurologic symptoms, fever, and skin lesions. A woman presented with shortness of breath and a chest CT scan showed diffuse interstitial thickening and ground glass opacities suggestive of an interstitial lung disease. On physical exam she was noted to have splenomegaly. The patient died and at autopsy was found to have an IVLBL in her lungs as well as nearly all her organs that were sampled. Although rare, IVLBL should be included in the differential diagnosis of interstitial lung disease and this case underscores the importance of the continuation of autopsies.

  13. Mantle Cell Lymphoma of Intestine Presenting as Multiple Lymphomatous Polyposis with Intussusception

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    Meena N. Jadhav

    2015-01-01

    Full Text Available Mantle Cell Lymphoma (MCL is a distinct clinicopathological subtype of B-cell non-Hodgkin's lymphoma (NHL accounting for 2-10% of all NHL cases. Gastrointestinal tract (GIT is the predominant site of extranodal MCL which commonly presents as Multiple Lymphomatous Polyposis (MLP. A 60 year old male presented with pain abdomen, diarrhea and weight loss of two months duration. On colonoscopy multiple polyps were found in the entire colon and rectum. Computed tomography revealed ileo-colic intussusception with nodularity in the lead point. Histopathology suggested features of MCL. On immunohistochemistry, the tumor cells were positive for CD20, CD5, Cyclin D1, negative for CD3, CD10, CD23, and CD45 RO

  14. Non-Hodgkin's Lymphoma Primarily Presenting with Fanconi Syndrome and Acute Kidney Injury

    Institute of Scientific and Technical Information of China (English)

    Wen-ling Ye; Bing Han; Bing-yan Liu; Chan Meng; Wei Ye; Yu-bing Wen; Hang Li; Xue-mei Li

    2010-01-01

    @@ KIDNEY involvement is common in non-Hodg-kin's lymphoma (NHL) with incidence up to 30%-40% in autopsy studies. However, it us-ually occurs late in the course of the disease and is clinically silent. Clinically overt renal disease in-cluding acute kidney injury (AKI) as its primary manifes-tation is rarely reported, moreover, Fanconi syndrome (FS) is extremely rare as the main manifestation in NHL. In this report, we presented a case of NHL primarily presenting with FS and AKI due to diffuse interstitial infiltration of NHL cells and emphasized the important role of renal biopsy, especially renal immunohistochemical analysis in the di-agnosis of renal diffuse lymphoma.

  15. Burkitt's non-Hodgkins lymphoma presenting as facial nerve palsy in HIV-positive patients.

    Science.gov (United States)

    Woodcock, H; Nelson, M

    2011-02-01

    An isolated facial nerve palsy is rare as the presentation of a central nervous system lymphoma. In this case series, we present the clinical features of three HIV-positive patients presenting with facial nerve palsies due to HIV-associated Burkitt's lymphoma. These patients had a non-resolving facial paralysis, which occurred during a late stage of HIV. Magnetic resonance imaging (MRI) did not show leptomeningeal enhancement. Cerebrospinal fluid revealed a lymphocytosis with elevated protein and low glucose levels. The diagnosis of Burkitt's lymphoma was made on histology which showed the characteristic 'starry sky' appearance due to scattered tangible body-laden macrophages. The patients were commenced on the intensive chemotherapy regimen of CODOX-M/IVAC. Two patients died of disease progression and the third patient died of chemotherapy toxicity. This case series highlights the need for a high index of suspicion for underlying malignancy when a patient presents with a persistent facial paralysis in the later stages of HIV infection.

  16. Febrile cholestatic disease as an initial presentation of nodular lymphocyte-predominant Hodgkin lymphoma

    Institute of Scientific and Technical Information of China (English)

    Anna; Mrzljak; Slavko; Gasparov; Ika; Kardum-Skelin; Vesna; Colic-Cvrlje; Slobodanka; Ostojic; Kolonic

    2010-01-01

    Febrile cholestatic liver disease is an extremely unusual presentation of Hodgkin lymphoma(HL).The liver biopsy of a 40-year-old man with febrile episodes and cholestatic laboratory pattern disclosed an uncommon subtype of HL,a nodular lymphocyte-predominant HL(NLPHL).Liver involvement in the early stage of the usually indolent NLPHL's clinical course suggests an aggressiveness and unfavorable outcome.Emphasizing a liver biopsy early in the diagnostic algorithm enables accurate diagnosis and appropriate tre...

  17. Minimal change disease: an unusual presentation of marginal zone MALT lymphoma.

    Science.gov (United States)

    Inayat, Faisal; Law, Jason K; Myers-Gurevitch, Patricia M; Seshan, Surya V; Perlman, Alan S

    2016-03-01

    Minimal change disease (MCD) in association with low-grade extra-nodal marginal zone B-cell lymphoma (MZL) of mucosa-associated lymphoid tissue (MALT) (MALT lymphoma) is a rare clinicopathologic entity. We report a 68-year-old male who presented with nephrotic range proteinuria as the first manifestation of underlying MZL, confirmed with standard set of investigations. Being a steroid non-responder, he was treated with rituximab demonstrating a marked response with resolution of proteinuria. However, he relapsed after 3 months. Upon relapse, a combination of rituximab and bendamustine (R-Benda) was initiated achieving sustained resolution of proteinuria. No additional treatment was administered and the proteinuria has remained in remission for over a year.

  18. Burkitt's lymphoma: a child's case presenting in the maxilla. Clinical and radiological aspects.

    Science.gov (United States)

    Valenzuela-Salas, Borja; Dean-Ferrer, Alicia; Alamillos-Granados, Francisco-Jesús

    2010-05-01

    Burkitt's lymphoma (BL) is a neoplasm which, despite its very aggressive behaviour is potentially curable. It typically affects the paediatric population. BL belongs to the non-Hodgkin lymphomas group, and is the first human tumour undoubtedly related to a viral origin (Epstein-Barr virus). Two main clinical subtypes are recognized: endemic or African type, and sporadic type; HIV associated BL constitutes a third type. Although common in endemic BL, maxillary involvement is rare in sporadic cases. This, together with the clinical lack of specificity associated to this location, makes diagnosis difficult. New chemotherapeutic protocols achieve a high survival rate. Most important prognostic factors are location and tumour stage. We report a paediatric case of BL presenting in the maxilla, with a review and a description of the characteristics of the disease.

  19. Extranodal NK/T-cell lymphoma presenting with primary cardiac involvement

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    Lisa M. Lepeak

    2011-08-01

    Full Text Available Primary cardiac lymphoma is extremely uncommon. We report a case of a 54 year old Caucasian male with a history of non-small cell lung cancer treated by surgical resection who presented with chest pain and dyspnea on exertion. Computerized tomography (CT imaging confirmed a 7.8¥3.8 cm right atrial soft tissue mass infiltrating the lateral wall of the right atrium, and a 5 cm pericardiophrenic mass. Echocardiography confirmed a moderate pericardial effusion without tamponade physiology. Percutaneous biopsy of the pericardiophrenic mass revealed pathologic features diagnostic of NK/T-cell lymphoma. He received CHOP chemotherapy with some improvement in symptoms, but experienced radiographic progression after 2 cycles. He received palliative involved field radiotherapy but developed new sites of progressive disease within the abdomen and died shortly after completing radiotherapy. NK/T-cell lymphomas are aggressive tumors that may present with unusual extranodal disease sites. Prompt diagnosis with consideration for referral to a specialty center with experience in treatment of these rare tumors may offer the greatest potential for improving treatment outcomes.

  20. Acute kidney injury and bilateral symmetrical enlargement of the kidneys as first presentation of B-cell lymphoblastic lymphoma.

    Science.gov (United States)

    Shi, Su-fang; Zhou, Fu-de; Zou, Wan-zhong; Wang, Hai-yan

    2012-12-01

    Lymphoblastic lymphoma is an uncommon subtype of lymphoid neoplasm in adults. Acute kidney injury at initial presentation due to lymphoblastic lymphoma infiltration of the kidneys has rarely been described. We report a 19-year-old woman who presented with acute kidney injury due to massive lymphomatous infiltration of the kidneys. The diagnosis of B-cell lymphoblastic lymphoma was established by immunohistochemical study of the biopsied kidney. The patient had an excellent response to the VDCLP protocol (vincristine, daunomycin, cyclophosphamide, asparaginase, and dexamethasone) with sustained remission. We recommend that lymphomatous infiltration be considered in patients presenting with unexplained acute kidney injury and enlarged kidneys.

  1. Mediastinal B-Cell Lymphoma Presenting with Jugular-Subclavian Deep Vein Thrombosis as the First Presentation

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    Sherif Ali Eltawansy

    2015-01-01

    Full Text Available Jugular venous thrombosis infrequently could be secondary to malignancy and has seldom been reported secondary to mediastinal large B-cell lymphomas. The postulated mechanisms are mechanical compression that leads to stagnation of blood in the venous system of the neck and/or an increase in the circulating thrombogenic elements that could cause venous thromboembolism as a paraneoplastic phenomenon. We report the case of a middle aged male presenting with right sided neck pain and arm swelling secondary to ipsilateral jugular-subclavian deep vein thrombosis. Investigations revealed it to be secondary to a mediastinal mass shown on CT scan of the chest.

  2. The Role of Endoscopic Ultrasound in Primary Pancreatic Lymphoma Presented with Acute Pancreatitis: A Case Report

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    Seyed Hassan Abedi

    2014-09-01

    Full Text Available Context Primary pancreatic lymphoma (PPL is rare but manageable malignant tumor of the pancreas which may be confused with pancreatic adenocarcinoma. Case report We report a 38 year-old smoker man with IVDA and history of B (inactive carrier, C (Ia genotype hepatitis and negative for HIV admitted to our hospital because of nausea, vomiting, epigastric and RUQ pain as a result of long period of alcohol consumption. Due to high amount of amylase (480 U/L and lipase (326 U/L Pancreatitis was diagnosed. Four days after admission CT was done that showed enhancement a large tumor of the head of the pancreas. Endoscopic ultrasound (EU revealed diffusely enlarged of pancreas. There was a mixed echoic mass lesion 37-50 mm at the head of pancreas (R/O walled off necrosis with adhesion to portal vein and SMV. On the other hand the CBD was 9 mm. Abdominal MRI and MRCP for patient was done. The intra-hepatic bile ducts, main hepatic ducts, CHD and CBD were mildly dilated and displaced to the right side by a large lobulated mass (160×112×130 mm of pancreatic head with low signal intensity on T1W images and high intensity on T2W images. We did EUS-FNA and there was a mixed echoic lesion (38-40mm adhered to head of pancreas with invasion to portal vein, SMA and SMV. The diagnosis of pancreatic lymphoma was established by immunohistochemical study and the assessment of the neoplastic cells revealed B cell lymphoma phenotypes. Conclusion Herein we present an interesting 38 year- old man with pancreas head tumor. Primary pancreatic lymphoma is almost never suspected clinically. It is usually diagnosed by imaging and histological methods.

  3. The role of endoscopic ultrasound in primary pancreatic lymphoma presented with acute pancreatitis: a case report.

    Science.gov (United States)

    Abedi, Seyed Hassan; Ahmadzadeh, Alireza; Nikmanesh, Ali; Mohammad Alizadeh, Amir Houshang

    2014-09-28

    Primary pancreatic lymphoma (PPL) is rare but manageable malignant tumor of the pancreas which may be confused with pancreatic adenocarcinoma. We report a 38 year-old smoker man with IVDA and history of B (inactive carrier), C (Ia genotype) hepatitis and negative for HIV admitted to our hospital because of nausea, vomiting, epigastric and RUQ pain as a result of long period of alcohol consumption. Due to high amount of amylase (480 U/L) and lipase (326 U/L) Pancreatitis was diagnosed. Four days after admission CT was done that showed enhancement a large tumor of the head of the pancreas. Endoscopic ultrasound (EU) revealed diffusely enlarged of pancreas. There was a mixed echoic mass lesion 37-50 mm at the head of pancreas (R/O walled off necrosis) with adhesion to portal vein and SMV. On the other hand the CBD was 9 mm. Abdominal MRI and MRCP for patient was done. The intra-hepatic bile ducts, main hepatic ducts, CHD and CBD were mildly dilated and displaced to the right side by a large lobulated mass (160×112×130 mm) of pancreatic head with low signal intensity on T1W images and high intensity on T2W images. we did EUS-FNA and there was a mixed echotic lesion (38-40 mm) adhered to head of pancreas with invasion to portal vein, SMA and SMV. The diagnosis of pancreatic lymphoma was established by immunohistochemical study and the assessment of the neoplastic cells revealed B cell lymphoma phenotypes. Herein we present an interesting 38 year- old man with pancreas head tumor. Primary pancreatic lymphoma is almost never suspected clinically. It is usually diagnosed by imaging and histological methods.

  4. Extranodal Marginal Zone Lymphoma Presenting within the Meckel Diverticulum as Diverticulitis: A Case Report

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    A. Nael

    2014-01-01

    Full Text Available Meckel diverticulum is the most common congenital defect of the gastrointestinal tract. It can be asymptomatic or mimic appendicitis and may be complicated by bleeding, diverticulitis, obstruction, and, rarely, neoplasia. We report the first case of extranodal marginal zone lymphoma occupying a Meckel diverticulum. A 44-year-old man with history of colonic diverticulitis presented to the emergency department for evaluation of acute abdominal pain. Radiography showed enteric obstruction, prompting diagnostic laparoscopy. Above the level of mid-ileum an intact Meckel diverticulum was identified. Microscopy showed extensive infiltration of sheets of small lymphocytes with abundant cytoplasm (monocytoid B-cells prominently in submucosa and focally transmural involving serosal adipose tissue with multiple reactive germinal centers. The immunostains showed positivity for CD20, BCL-2, and CD43 (weak and negativity for CD3, CD5, BCL-1, CD10, and BCL-6 in monocytoid B-cells. Fluorescence in situ hybridization studies revealed API2-MALT1 fusion signals consistent with t(11;18(q21;q21, which confirmed the diagnosis of extranodal marginal zone lymphoma, also known as mucosa associated lymphoid tissue lymphoma.

  5. Prognosis Factors in Patients with Non-Hodgkin’s Lymphoma Presenting with Gastrointestinal Tract Symptoms

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    Brian H Weinerman

    1991-01-01

    Full Text Available Forty-one individuals who presented with symptoms referable to the gastrointestinal tract were extracted from the authors’ total review of non-Hodgkin’s lymphoma in Manitoba from 1968 through 1978. Only cases at stages I to III were included, and there were 22 males and 19 females. Sixteen non-Hodgkin’s lymphomas arose in the small bowel, 15 in the stomach and 10 in the colon. The natural history of this group of gastrointestinal non-Hodgkin’s disease is described, and univariate and multivariate analyses were done using the variables of sex, pathology, stage, resection, site of disease and initial chemotherapy. The median survival of the group was 28 months. Sex and stage appeared to be important prognostically, but after multivariate analysis, only the female sex appear to be a good prognostic variable. There was a suggestion that resection should be attempted in these lesions, but there was not a large sample size in this group. In addition, it was felt that this group of individuals followed the same survival pattern as did poor prognosis non-Hodgkin’s lymphomas of nongastrointestinal origin.

  6. Nasal Type Extranodal Natural Killer/T (NK/T) Cell Lymphoma Presenting as Periorbital Cellulitis: A Case Report

    Science.gov (United States)

    Shawabkeh, Ma’in Ali Al; Sulaiti, Mansour Al; Sa’ey, Hamad Al; Ganesan, Shanmugam

    2016-01-01

    Patient: Male, 25 Final Diagnosis: Nasal type • extra nodal NK/T-cell lymphoma Symptoms: Left periorbital swelling • redness • pain for 25 days • yellowish eye discharge associated • headache • fever Medication: — Clinical Procedure: — Specialty: Otolaryngology Objective: Unusual clinical course Background: Extranodal lymphoma of the paranasal sinuses is a rare clinical entity seen in only 5–8% of extranodal lymphomas of the head and neck. Nasal natural killer/T cell lymphoma (Nasal NKTCL), which is a subtype of peripheral T cell lymphoma, constitutes about 1.4% of all lymphomas. NKTCL is usually diagnosed at a late stage because it presents with nonspecific symptoms in the early stages. Case Report: We report the case of a 25-year-old male patient who presented with periorbital swelling treated as fungal sinusitis but proven to have NKTCL. We review the literature and discuss the clinical manifestations of the disease, its relation to EBV virus, the histological and radiological characteristics, the prognostic indicators, and treatment options. This case report shows physicians that NKTCL lymphoma can present as periorbital cellulitis, although few similar cases are found in the literature. Conclusions: NKTCL is a destructive midline tumor that should be kept in mind as a differential diagnosis of paranasal sinus lesions to help in early diagnosis, which can improve the prognosis. PMID:27932776

  7. Presentation of Chronic Lymphocytic Leukemia/Small Lymphocytic Lymphoma in a Warthin Tumor: Case Report and Literature Review.

    Science.gov (United States)

    Jawad, Hadeel; McCarthy, Peter; O'Leary, Gerard; Heffron, Cynthia C

    2017-10-01

    Warthin tumor is the second most common salivary gland neoplasm. It occurs more commonly in males than in females. Malignant transformation in Warthin tumor is a rare but well-recognized phenomenon; however, the development or presentation of lymphoma in a Warthin tumor is rare. An 80-year-old man presented with painless mass of the right parotid gland of 2 years duration with recent ulceration of the overlying skin and right cervical lymphadenopathy underwent a surgical resection of parotid mass and biopsy of the periglandular lymph nodes. The histological diagnosis was malignant lymphoma, chronic lymphocytic leukemia/small lymphocytic lymphoma, present within the stroma of a Warthin tumor, and also present within the adjacent lymph node. This case is the third reported case describing a collision of Warthin tumor and chronic lymphocytic leukemia/small lymphocytic lymphoma. It also emphasizes the importance of careful examination of the lymphoid stroma of these tumors.

  8. Ill-fitting dentures as primary presentation of mantle cell lymphoma: A case report and literature review of the primary mantle cell lymphomas of the hard palate

    Directory of Open Access Journals (Sweden)

    Ömür Dereci

    2015-01-01

    Full Text Available Mantle cell lymphoma (MCL is a subtype of B-cell non-Hodgkin′s lymphoma seen predominantly in males. Common extra-nodal sites of involvement of MCL are Waldeyer′s ring, gastrointestinal tract, bone marrow and peripheral blood. The extra-nodal palatal localization of MCL is quite uncommon. MCL is seen in predominantly older patients, therefore undiagnosed MCL patients are likely to have total prosthesis. In this study, a case of MCL, initially presenting as palatal swelling was reported with relevant literature review and the possible role of dental professionals in the diagnosis of this rare entity was discussed.

  9. Radiotherapy Compared to Other Strategies in the Treatment of Stage I/II Follicular Lymphoma: A Study of 404 Patients with a Median Follow-Up of 15 Years.

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    Dlawer Abdulla Barzenje

    Full Text Available To investigate outcome for patients with follicular lymphoma (FL stage I-II treated at a population-based referral institution with a median follow-up of 15 years. Overall and cause-specific survival was compared to that of a sex, age and residency matched individuals from normal population.404 patients with early stage FL treated between 1980 and 2005 were retrospectively analyzed. Two of three patients had stage I disease. Based on clinical characteristics, first line treatments were radiotherapy (RT (48% of patients, chemotherapy (CT (16%, combined chemo-and radiotherapy (CRT (16% or observation (OBS (15%. Survival was modeled with Kaplan-Meier methodology. Multivariate analyses were performed with the Cox model.Fifteen years overall survival (OS, progression free survival (PFS and time to next treatment (TNT were 50% (95% confidence interval [CI]: 45-55, 42% (95% CI: 36-47 and 48% (95% CI, 42-54, respectively. For patients treated with RT 97% achieved a complete remission, and 15 year OS, PFS and TNT were 57% (95% CI, 50-64, 46% (95% CI, 39-54 and 49% (95% CI, 42-57, respectively. Relapse rate after RT and CRT was 49% and 36%, respectively. Only 2% of patients who received RT or CRT relapsed inside the radiation field and 5% had isolated near-field relapse. No statistical differences were found between treatment groups regarding death from cardiovascular disease or incidence of second cancer. Compared to a matched normal population, non-lymphoma cancer mortality was higher among patients given RT, hazard ratio 1.66 (95% CI: 1.14-2.42; P<0.01. Compared to other treatment modalities, patients selected for observation without treatment did not have inferior outcome.A differentiated treatment strategy in early stage FL results in long term survival for the majority of patients. OBS is a valid initial choice for selected patients without lymphoma-related symptoms.

  10. Genetically Modified T-cell Infusion Following Peripheral Blood Stem Cell Transplant in Treating Patients With Recurrent or High-Risk Non-Hodgkin Lymphoma

    Science.gov (United States)

    2017-01-27

    Adult Grade III Lymphomatoid Granulomatosis; Cutaneous B-cell Non-Hodgkin Lymphoma; Extranodal Marginal Zone B-cell Lymphoma of Mucosa-associated Lymphoid Tissue; Intraocular Lymphoma; Nodal Marginal Zone B-cell Lymphoma; Post-transplant Lymphoproliferative Disorder; Recurrent Adult Burkitt Lymphoma; Recurrent Adult Diffuse Large Cell Lymphoma; Recurrent Adult Diffuse Mixed Cell Lymphoma; Recurrent Adult Diffuse Small Cleaved Cell Lymphoma; Recurrent Adult Grade III Lymphomatoid Granulomatosis; Recurrent Adult Immunoblastic Large Cell Lymphoma; Recurrent Adult Lymphoblastic Lymphoma; Recurrent Grade 1 Follicular Lymphoma; Recurrent Grade 2 Follicular Lymphoma; Recurrent Grade 3 Follicular Lymphoma; Recurrent Mantle Cell Lymphoma; Recurrent Marginal Zone Lymphoma; Recurrent Small Lymphocytic Lymphoma; Refractory Hairy Cell Leukemia; Small Intestine Lymphoma; Splenic Marginal Zone Lymphoma; Testicular Lymphoma; Waldenström Macroglobulinemia

  11. An Unusual Presentation of Liver Failure in a Patient with Primary Gastrointestinal Hodgkin's Lymphoma

    Directory of Open Access Journals (Sweden)

    Gabrielle B. Rocque

    2011-01-01

    Full Text Available Introduction. Hodgkin's lymphoma (HL presenting either with primary bowel involvement or with cholestasis is unusual. The combination of primary gastrointestinal HL presenting with cholestasis and ductopenia has not been previously described. Case Report. We present a case of primary gastrointestinal HL with evidence of liver involvement, but also with prominent ductopenia on liver biopsy and associated intrahepatic cholestasis. A 50-year-old man with a history of Crohn's disease presented with a bowel obstruction, for which he underwent a small bowel resection. Histology revealed HL. His course was complicated by cholestatic liver failure. A subsequent liver biopsy revealed both focal involvement by lymphoma and ductopenia, resembling vanishing bile duct syndrome (VBDS. He was treated with chemotherapy with improvement in his cholestasis, but he eventually succumbed due to further complications of his disease and treatment toxicities. Conclusion. This case of primary gastrointestinal HL associated with ductopenia does not meet classic criteria for VBDS, but the clinical presentation and pathology are suggestive of a VBDS-like paraneoplastic process. Therapies for HL in the setting of cholestatic liver failure require special consideration, but some reports of durable remissions and recovery of liver function have been reported.

  12. Uncommon presentation of Burkitt's lymphoma: a case report; Apresentacao atipica do linfoma de Burkitt: relato de caso

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    Caldeira Junior, Sandro Ferreira; Torres, Lucas Rios; Rogerio, Ricardo Mendes; Macedo, Leonardo Lopes de; Ferri, Francisco; Souza, Ricardo Pires de [Hospital Heliopolis, Sao Paulo, SP (Brazil). Servico de Radiologia e Diagnostico por Imagem

    2007-01-15

    Burkitt's lymphoma is a rapidly proliferating, highly aggressive B-cell lymphoma of non-Hodgkin subtype. We present a case of a young adult with weight loss and abdominal mass. A computed tomography of abdomen showed a bulky abdominal mass with calcifications. It has not previously been done any type of treatment. The diagnostic was made by pathology and immunohistochemistry. (author)

  13. HIV-Infected Spleens Present Altered Follicular Helper T Cell (Tfh Subsets and Skewed B Cell Maturation.

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    Lucie Colineau

    Full Text Available Follicular helper T (Tfh cells within secondary lymphoid organs control multiple steps of B cell maturation and antibody (Ab production. HIV-1 infection is associated with an altered B cell differentiation and Tfh isolated from lymph nodes of HIV-infected (HIV+ individuals provide inadequate B cell help in vitro. However, the mechanisms underlying this impairment of Tfh function are not fully defined. Using a unique collection of splenocytes, we compared the frequency, phenotype and transcriptome of Tfh subsets in spleens from HIV negative (HIV- and HIV+ subjects. We observed an increase of CXCR5+PD-1highCD57-Tfh and germinal center (GC CD57+ Tfh in HIV+ spleens. Both subsets showed a reduced mRNA expression of the transcription factor STAT-3, co-stimulatory, regulatory and signal transduction molecules as compared to HIV- spleens. Similarly, Foxp3 expressing follicular regulatory T (Tfr cells were increased, suggesting sustained GC reactions in chronically HIV+ spleens. As a consequence, GC B cell populations were expanded, however, complete maturation into memory B cells was reduced in HIV+ spleens where we evidenced a compromised production of B cell-activating cytokines such as IL-4 and IL-10. Collectively our data indicate that, although Tfh proliferation and GC reactions seem to be ongoing in HIV-infected spleens, Tfh "differentiation" and expression of costimulatory molecules is skewed with a profound effect on B cell maturation.

  14. Usefulness of Random Skin Biopsy as a Diagnostic Tool of Intravascular Lymphoma Presenting With Fever of Unknown Origin.

    Science.gov (United States)

    Pongpudpunth, Marinya; Rattanakaemakorn, Ploysyne; Fleischer, Alan B

    2015-09-01

    Intravascular B-cell lymphoma (IVBCL) is a rare type of extranodal lymphoma in which the lymphoma proliferates within vascular lumen. One of the most common presenting symptoms is prolonged fever. Although examination of a random skin biopsy from healthy-appearing skin in patients with suspected intravascular lymphoma has been reported to be useful, the sensitivity of this method for diagnosis is still unknown. To evaluate the usefulness of a random skin biopsy in the diagnosis of intravascular lymphoma in the patient presenting with fever of unknown origin. All cases that we performed random skin biopsy were retrospectively reviewed from March 2007 to June 2012 in Ramathibodi Hospital. The incisional biopsy technique was performed in 3 separate locations including 2 specimens from both upper thighs and 1 specimen from the abdomen. Of the 24 cases that were consulted to dermatologist for random skin biopsy, 3 cases (13%) were diagnosed of intravascular lymphoma and all presented with prolong fever, weight loss, and remarkable deterioration in Eastern Cooperative Oncology Group (ECOG) performance status (ECOG score) > 2. Interestingly, 2 cases (8%) were diagnosed with cytophagic histiocytic panniculitis from normal-appearing skin and subsequently investigated, which leads to the diagnosed of T-cell lymphoma. In an elderly patient with fever of unknown origin and/or weight loss with no evident of skin lesion, markedly elevated lactate dehydrogenase, remarkable deterioration in ECOG performance status who suspected IVBCL should be consider to perform incisional random skin biopsy at least 3 specimens from both thighs and abdomen to achieve a high sensitivity. We propose that this technique may serve as an important diagnostic tool to obtain the diagnosis of IVBCL. In summary, 5 of 24 (21%) positive results from random skin biopsy helped diagnoses of lymphoma.

  15. Castleman's disease in the long term follow-up of a patient with non Hodgkin's lymphoma: An unusual presentation.

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    Rajjyoti Das

    2014-09-01

    Full Text Available Castleman's disease is a benign condition characterized by localized or generalized lymphadenopathy. It is an inherited disorder and usually seen concurrently with lymphomas. We present here a case of multi-centric hyaline vascular type of Castleman's disease detected in the long term follow-up of a patient who was being previously treated for non Hodgkin's lymphoma. In the long term follow-up of patient with lymphomas it can be a cause of lymph node enlargement bearing a clinical resemblance to recurrence of lymphoma. The diagnosis of should be made by a combination of clinical, radiological examination and histopathological examination with immunohistochemistry study. [Natl J Med Res 2014; 4(3.000: 259-261

  16. HIV-associated primary effusion lymphoma presenting as a paracardial mass

    Science.gov (United States)

    Katz, Heather; Rose, Cielo; Rivera, Nina Thakker; Bray, Natasha

    2015-01-01

    Primary effusion lymphoma (PEL), a rare type of non-Hodgkin's lymphoma, is an AIDS-defining illness and always associated with human herpesvirus 8 (HHV-8). Classic presentations involve the pleural, pericardial or peritoneal cavities. Infrequently, extracavitary solid tumours develop. Treatment of PEL requires chemotherapy and highly active antiretroviral therapy (HAART). We report a case of a 46-year-old man, who presented with right-sided chest pain, dyspnoea and night sweats. Evaluation revealed decreased breath sounds and dullness to percussion on the right side of the chest. Imaging demonstrated a 6.1 cm×6.3 cm right paracardial mass and right-sided pleural effusion. Pleural fluid was HHV-8 positive. The patient was diagnosed with PEL with extracavitary involvement and treated with chemotherapy and concurrent HAART. This case is the first reported case of extracavitary paracardial involvement and adds new insight to the accepted treatment for PEL with extracavitary lesions. PMID:25725030

  17. Hemosiderin laden macrophages and hemosiderin within follicular cells distinguish benign follicular lesions from follicular neoplasms

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    Jaffar Reema

    2009-01-01

    Full Text Available Background: Published criteria to distinguish benign colloid nodules from follicular neoplasms emphasize only three interdependent features: size of follicles, amount of colloid, and cellularity. There is a need for the validation of other independent criteria. Methods: This study quantified the significance of cystic change, defined as presence of macrophages, and the presence of hemosiderin in either the macrophages or follicular cells. The cohort consisted of 165 patients with fine needle aspiration (FNA and histologic follow-up of either goiter (101, follicular adenoma (47, or follicular carcinoma (17. Papillary thyroid carcinomas and Hürthle cell neoplasms were excluded from the cohort, because these categories are known to show cystic change and hemosiderin. FNAs were reviewed blindly with the most cellular slide scored for the presence of macrophages and/or hemosiderin. Results: Hemosiderin within macrophages were seen in 67% (68 of 101 of the goiters and only 6% (four of 64 of follicular neoplasms ( P < .0001. All four follicular neoplasms with hemosiderin in macrophages were adenomas. Three of these four had equivocal features of a benign colloid nodule histologically. None of the 17 follicular carcinomas had hemosiderin in macrophages ( P < .12. Macrophages without hemosiderin also strongly distinguished goiters from neoplasms (83% vs 17% but appears less useful as a criterion since macrophages were present within 3 of 17 follicular carcinomas. Hemosiderin within follicular epithelial cells was present in 18% (18 of 101 of goiters, whereas none of the 64 follicular neoplasms had intraepithelial hemosiderin ( P < .0003. Conclusions: If papillary thyroid carcinoma and Hürthle cell neoplasm are ruled out, our findings indicate that the presence of hemosiderin virtually excludes a clinically significant follicular neoplasm.

  18. Sinonasal Lymphoma Presenting as a Probable Sanctuary Site for Relapsed B Acute Lymphoblastic Leukaemia: A Case Report and Review of the Literature

    Directory of Open Access Journals (Sweden)

    W. Y. Lim

    2015-01-01

    Full Text Available Sinonasal lymphoma is a non-Hodgkin lymphoma (NHL representing 1.5% of all lymphomas. It presents as an unremitting ulceration with progressive destruction of midline sinonasal and surrounding structures. Poor prognosis warrants early treatment although diagnosis is challenging and frequently delayed. It is usually primary in origin and to our knowledge the sinonasal region has never been reported as a sanctuary site in leukaemia/lymphoma relapse. We present a unique case of B-cell ALL (acute lymphoblastic leukaemia with late relapse to the nasal septum as a sinonasal lymphoblastic lymphoma and with genetic support for this as a sanctuary site.

  19. Mantle cell lymphoma of the gastrointestinal tract presenting with multiple intussusceptions – case report and review of literature

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    Abo Stephen M

    2009-07-01

    Full Text Available Abstract Background Mantle cell lymphoma (MCL is an aggressive type of B-cell non-Hodgkin's lymphoma that originates from small to medium sized lymphocytes located in the mantle zone of the lymph node. Extra nodal involvement is present in the majority of cases, with a peculiar tendency to invade the gastro-intestinal tract in the form of multiple lymphomatous polyposis. MCL can be accurately diagnosed with the use of the highly specific marker Cyclin D1. Few cases of mantle cell lymphoma presenting with intussuception have been reported. Here we present a rare case of multiple intussusceptions caused by mantle cell lymphoma and review the literature of this disease. Case presentation A 68-year-old male presented with pain, tenderness in the right lower abdomen, associated with nausea and non-bilious vomiting. CT scan of abdomen revealed ileo-colic intussusception. Laparoscopy confirmed multiple intussusceptions involving ileo-colic and ileo-ileal segments of gastrointestinal tract. A laparoscopically assisted right hemicolectomy and extended ileal resection was performed. Postoperative recovery was uneventful. The histology and immuno-histochemistry of the excised small and large bowel revealed mantle cell lymphoma with multiple lymphomatous polyposis and positivity to Cyclin D1 marker. The patient was successfully treated with Rituximab-CHOP chemotherapy and remains in complete remission at one-year follow-up. Conclusion This is a rare case of intestinal lymphomatous polyposis due to mantle cell lymphoma presenting with multiple small bowel intussusceptions. Our case highlights laparoscopic-assisted bowel resection as a potential and feasible option in the multi-disciplinary treatment of mantle cell lymphoma.

  20. Salvia Hispanica Seed in Reducing Risk of Disease Recurrence in Patients With Non-Hodgkin Lymphoma

    Science.gov (United States)

    2017-01-26

    Adult Nasal Type Extranodal NK/T-Cell Lymphoma; Adult T-Cell Leukemia/Lymphoma; Anaplastic Large Cell Lymphoma; Angioimmunoblastic T-Cell Lymphoma; B Lymphoblastic Leukemia/Lymphoma; Blastic Plasmacytoid Dendritic Cell Neoplasm; Burkitt Leukemia; Central Nervous System Lymphoma; Chronic Lymphocytic Leukemia/Small Lymphocytic Lymphoma; Diffuse Large B-Cell Lymphoma; Enteropathy-Associated T-Cell Lymphoma; Extranodal Marginal Zone Lymphoma of Mucosa-Associated Lymphoid Tissue; Grade 1 Follicular Lymphoma; Grade 2 Follicular Lymphoma; Grade 3 Follicular Lymphoma; Hepatosplenic T-Cell Lymphoma; Lymphoplasmacytic Lymphoma; Mantle Cell Lymphoma; Mediastinal (Thymic) Large B-Cell Lymphoma; Mycosis Fungoides; Nasal Type Extranodal NK/T-Cell Lymphoma; Nodal Marginal Zone Lymphoma; Peripheral T-Cell Lymphoma, Not Otherwise Specified; Post-Transplant Lymphoproliferative Disorder; Primary Cutaneous Anaplastic Large Cell Lymphoma; Primary Effusion Lymphoma; Sezary Syndrome; Splenic Marginal Zone Lymphoma; Subcutaneous Panniculitis-Like T-Cell Lymphoma; Systemic Anaplastic Large Cell Lymphoma; T Lymphoblastic Leukemia/Lymphoma; Transformed Recurrent Non-Hodgkin Lymphoma

  1. AIDS-associated plasmablastic lymphoma presenting as a poorly differentiated esophageal tumor: A diagnostic dilemma

    Institute of Scientific and Technical Information of China (English)

    2008-01-01

    Plasmablastic lymphoma (PBL) is a rare form of diffuse large B-cell lymphoma characterized by weak/absent expression of conventional B-cell markers and strong expression of plasma cell markers. It is strongly associated with human immunodeficiency virus (HIV) and Epstein Barr virus infection, and shows an unusual tropism to the oral cavity. Herein we describe a patient with AIDS who presented with weight loss and dysphagia owing to a large gastroesophageal mass. His radiographic and endoscopic findings and long history of cigarette consumption suggested carcinoma. Biopsy demonstrated a poorly differentiated tumor stained negatively to routine lymphoid markers including CD20. However, gene rearrangement studies confirmed a B-cell process and a more detailed immunohistochemical analysis revealed the cells stained positively for CD138 (plasma cell antigen). These findings were diagnostic of PBL. Our report reviews the wide differential diagnosis of PBL and underscores the importance of a broad array of viral and molecular studies needed to establish this diagnosis.

  2. 68. Unusual presentation of mediastinal lymphoma and role of cardiac MRI

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    N. Mahmood

    2016-07-01

    Full Text Available Diffuse large B cell lymphoma (DLBCL is the most common histologic subtype of non-Hodgkin lymphoma (NHL accounting for approximately 25% of NHL cases. One of the common subtype of DLBCL is primary DLBCL of the mediastinum. Case report this 65 year old female known to have diabetes and hypertension presented to our emergency department with history of epigastric pain for last 2 h. Her electrocardiogram (ECG showed right bundle block with left posterior hemi-block representing bi-fasicular block with minimal ST segment depression in leads V4–V6. Her initial routine laboratory results revealed normal renal function, blood counts and liver profile. Her cardiac bio-markers were elevated with Troponin I of 1.22 and CPK of 35. She was admitted by the cardiology team diagnosis of non-ST elevation myocardial infarction (NSTEMI. She was started with usual anti-ischemic. Next morning she had echocardiography which revealed a large mediastinal mass on the antero-lateral aspect of the left ventricle infiltrating the basal lateral and anterior wall. This mass was encasing the origin of the great vessels and also infiltrating the left atrium occluding the Left atrial appendage and left upper pulmonary vein. It was infiltrating the Right ventricular outflow tract causing obstruction to the flow with a gradient of 52 mmHg. Cardiac MRI showed multiple cardiac masses, the largest of which was originating from the anterior mediastinum and going posteriorly then infiltrating RV and within the right ventricular out-flow tract (RVOT causing significant obstruction. The magnetic resonance characteristics of the intra cardiac and extra cardiac masses were same and with features of central necrosis was highly suggestive of lymphoma. The CT scan of the chest and abdomen showed the same cardiac findings as of cardiac MRI and multiple enlarged thoracic, retroperitoneal, left common iliac lymph nodes. Patient had CT guided Lymph node biopsy from the mediastinal lymph

  3. Uncommon Presentation of Isolated Jejunal Lymphoma Masquerading as Crohn’s Disease

    Directory of Open Access Journals (Sweden)

    Swati Sattavan

    2016-01-01

    Full Text Available Primary gastrointestinal lymphoma is a rare entity, commonly involving stomach, small bowel, and colorectum. The usual location for small bowel B cell lymphoma is distal ileum due to abundant lymphoid tissue. We are reporting the case of a 53-year-old lady presumptively diagnosed as Crohn’s disease on clinical and radiological grounds but histopathologically proven to be an unusual variant of isolated primary non-Hodgkin’s lymphoma.

  4. Clinical presentation and endoscopic features of primary gastric Burkitt lymphoma in childhood, presenting as a protein-losing enteropathy: a case report

    Directory of Open Access Journals (Sweden)

    Chieng Jenny Hui Chia

    2009-06-01

    Full Text Available Abstract Introduction Burkitt lymphoma and B cell lymphomas in childhood may arise in many atypical locations, which on rare occasions can include gastric mucosa. A case of primary gastric Burkitt lymphoma is described in a child presenting as a protein-losing enteropathy, including the direct monitoring of the disease response by sequential endoscopic biopsy and molecular analysis. Case presentation We report a 9-year-old boy who presented with gross oedema, ascites and respiratory distress caused by a protein-losing enteropathy. Initial imaging investigations were non-diagnostic but gastroduodenal endoscopy revealed massive involvement of the gastric mucosa with a primary Burkitt lymphoma. His subsequent clinical progress and disease response were monitored directly by endoscopy and he remains in clinical remission 4 years after initial diagnosis. Conclusions This is the first case report of primary Burkitt lymphoma presenting as a protein-losing enteropathy. The clinical course and progress of the patient were monitored by sequential endoscopic biopsy, histology and molecular analysis by fluorescence in situ hybridisation.

  5. Disseminated non-Hodgkin's lymphoma presenting as bilateral salivary gland enlargement: a case report

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    Revanappa, Manjunatha M. [Dept. of Oral Medicine and Radiology, College of Dental Sciences, Davangere (India); Sattur, Atul P.; Naikmasur, Venkatesh G. [Dept. of Oral Medicine and Radiology, SDM College of Dental Sciences and Hospital, Dharwad (India); Thakur, Arpita Rai [Dept. of Oral Medicine and Radiology, Jamia Milia Islamia University, New Delhi (India)

    2013-03-15

    Non-Hodgkin's lymphoma (NHL) constitutes a group of malignancies those arises from cellular components of lymphoid or extranodal tissues. The head and neck is the most common area for the presentation of these lymphoproliferative disorders. Primary involvement of salivary glands is uncommon. This report described a case of a 73-year-old female patient who presented with involvement of both nodal and extranodal sites, with predominant involvement of salivary glands. The tumor staging worked up along with imaging, histopathological, and immunohistochemical findings were discussed. Computed tomographic images showed the involvement of Waldeyer's ring, larynx, orbit, and spleen. This report described imaging and prognostic tumor markers in diagnosing, treatment planning, and prognosis.

  6. Cardiac Tamponade Associated with the Presentation of Anaplastic Large Cell Lymphoma in a 2-Year-Old Child

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    Gema Mira-Perceval Juan

    2015-01-01

    Full Text Available The anaplastic large cell lymphoma is a rare entity in pediatric patients. We present an unusual case of pericardial involvement, quite uncommon as extranodal presentation of this type of disorder, that provoked a life-risk situation requiring an urgent pericardiocentesis. To our knowledge, this is the first report on a child with pericardial involvement without an associated cardiac mass secondary to anaplastic large cell lymphoma in pediatric age. We report the case of a 21-month-old Caucasian male infant with cardiac tamponade associated with the presentation of anaplastic large cell lymphoma. Initially, the child presented with 24-day prolonged fever syndrome, cutaneous lesions associated with hepatomegaly, inguinal adenopathies, and pneumonia. After a 21-day asymptomatic period, polypnea and tachycardia were detected in a clinical check-up. Chest X-ray revealed a remarkable increase of the cardiothoracic index. The anaplastic large cell lymphoma has a high incidence of extranodal involvement but myocardial or pericardial involvements are rare. For this reason, we recommend a close monitoring of patients with a differential diagnosis of anaplastic large cell lymphoma.

  7. Mucosa associated lymphoid tissue lymphoma presenting within a solitary anti-mesenteric dilated segment of ileum: a case report

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    Storey Rowland

    2009-01-01

    Full Text Available Abstract Introduction Mucosa associated lymphoid tissue (MALT lymphoma is the third most common non-Hodgkin's lymphoma subtype. Clinical presentation is often insidious as a low-grade lesion and disease tends to remain localised for a long period of time. Ileal involvement is rare and presentation within an area of focal anti-mesenteric ileal wall dilation simulating a large diverticulum has not been reported. Case presentation A 59-year-old man of Caucasian origin presented to a general surgical outpatients clinic with an 18-month history of intermittent upper abdominal pain following meals. Following normal gastroscopy and abdominal ultrasound, a focally dilated segment of ileum was seen on computed tomography and further clarified by barium investigation. Histology of this segment demonstrated MALT lymphoma of the small bowel. Conclusion A solitary focally dilated segment of ileal wall may be neoplastic in nature and surgical resection needs to be considered.

  8. Brilliant cresyl blue staining does not present cytotoxic effects on human luteinized follicular cells, according to gene/protein expression, as well as to cytotoxicity tests.

    Science.gov (United States)

    Alcoba, Diego Duarte; Schneider, Júlia; Arruda, Letícia; Martiny, Patrícia Borba; Capp, Edison; von Eye Corleta, Helena; Brum, Ilma Simoni

    2017-03-01

    In vitro oocyte maturation presents many advantages and its success is related to the selection of fully grown oocytes. In animal models, staining of cumulus-oocyte complexes (COCs) with Brilliant Cresyl Blue (BCB) is widely used for this selection. However, a lack of information about the safety of BCB makes its applicability in humans questionable. Because granulosa and cumulus cells have a close relationship with the oocyte and taking into account that follicular cells are also exposed to BCB when this pre-selection method is applied, we aimed to evaluate the effects of BCB on human follicular cells exposed to BCB. Cytotoxicity tests (Sulforhodamine B and Neutral Red Uptake) and gene and protein expression of elements related to the cell cycle (BAX, BCL2, TP53 and CDKN1A), as well as to cell death and metabolism (GAPDH), glucose consumption, and estradiol and progesterone secretion, were examined in granulosa and cumulus cells with and without exposure to BCB. Regardless estradiol secretion and glucose consumption, all other evaluations presented similar results between control and treated (BCB) groups, independently of cell type. Therefore, our results demonstrate convincingly that BCB seems to be safe for use in humans and it should encourage future studies to evaluate the development of embryos derived from human oocytes selected by BCB staining. Copyright © 2016. Published by Elsevier Urban & Partner Sp. z o.o.

  9. Medial rectus muscle myositis as an atypical presentation of mucosa-associated lymphatic tissue lymphoma: a case report

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    Juliana Sá Freire Medrado Dias

    2014-04-01

    Full Text Available Here we describe the rare case of a 55-year-old man with medial rectus muscle myositis as an atypical presentation of non-Hodgkin B-cell mucosa-associated lymphoma (MALT. Pathology and immunohistochemistry of the affected muscle confirmed the diagnosis of a neoplasm. The primary etiology of orbital myositis is Graves' ophthalmopathy, but several other diseases may cause this clinical presentation. Therefore, the neoplastic causes must be eliminated from the differential diagnoses. non-Hodgkin B-cell mucosa-associated lymphoma is the most common histological type of lymphoma in the orbit, with the conjunctiva and lacrimal glands being the most commonly affected sites. However, it may also present in atypical forms involving others sites and tissues.

  10. Non-Hodgkin’s Lymphoma Presenting as Constrictive Pericarditis: A Rare Case Report

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    Maryam Nabati

    2016-10-01

    Full Text Available Constrictive pericarditis (CP is an uncommon post inflammatory disorder. It is described as pericardial thickening, myocardial constriction, and impaired diastolic filling. The most common etiologies are idiopathy, mediastinal radiotherapy, and prior cardiac surgery. Less common etiologies include viral infections, collagen vascular disorders, renal failure, sarcoidosis, tuberculosis, and blunt chest trauma. CP can less commonly be caused by malignancy. We report a very rare case of non-Hodgkin’s lymphoma (NHL presenting twice with attacks of decompensated heart failure. Echocardiography revealed that CP was responsible for the patient's symptoms as the first manifestation of NHL. Chest computed tomography scan and biopsy findings were compatible with the diagnosis of NHL. The patient received R-CHOP (cyclophosphamide, hydroxydaunorubicin, Oncovin®, and prednisone or prednisolone, combined with the monoclonal antibody rituximab chemotherapy. Three months later, there was significant improvement in the patient’s symptoms and considerable decrease in pericardial thickness.

  11. Endemic Burkitt Lymphoma: Long-term Outcome in 87 Patients Who Presented With Paraplegia in Cameroon.

    Science.gov (United States)

    Hesseling, P B; Mbah, G; Kouya, F; Kimbi, C; Nfor, P; Kaah, J; Kuruvilla, R; Best, A; Wharin, P

    2015-01-01

    The reported long-term outcome of endemic Burkitt lymphoma (eBL) patients who present with paraplegia is largely unknown. Records of BL patients treated with comparable short-interval cyclophosphamide chemotherapy schedules between 2004 and 2014 at three Baptist mission hospitals in Cameroon were reviewed. Survivors were followed up and examined at home or in hospital. Eighty-seven of 948 (9.2%) patients had paraplegia at diagnosis. The survival rate in eBL patients with paraplegia at diagnosis was 33% (n = 29) after follow-up of between 2 and 96 (median 40) months. Seven patients (24%) had neurological sequelae and needed rehabilitation. There was no relationship between the duration of symptoms (4 weeks) and the survival rate or the risk to have neurological sequelae. The survival rate and risk for sequelae were similar in patients with confirmed St. Jude stage III and IV diseases.

  12. First clinical use of ofatumumab, a novel fully human anti-CD20 monoclonal antibody in relapsed or refractory follicular lymphoma

    DEFF Research Database (Denmark)

    Hagenbeek, Anton; Gadeberg, Ole Vestergaard; Johnson, Peter

    2008-01-01

    Ofatumumab is a unique monoclonal antibody that targets a distinct small loop epitope on the CD20 molecule. Preclinical data show that ofatumumab is active against B-cell lymphoma/chronic lymphocytic leukemia cells with low CD20-antigen density and high expression of complement inhibitory molecules...... and profound B-cell depletion, and 65% of patients reverted to negative BCL2 status. Clinical response rates ranged from 20% to 63%. Median time to progression for all patients/responders was 8.8/32.6 months, and median duration of response was 29.9 months at a median/maximum follow-up of 9.2/38.6 months...

  13. Primary Diffuse Large B-Cell Lymphoma Localized to the Lacrimal Sac: A Case Presentation and Review of the Literature.

    Science.gov (United States)

    Zarrabi, Kevin; Desai, Ved; Yim, Brandom; Gabig, Theodore G

    2016-01-01

    We report a rare case of diffuse large B-cell lymphoma (DLBCL) of the lacrimal sac in a 50-year-old male. The incidence of primary ocular lymphoma is low and it is considered a rare disease. Moreover, reports of ocular DLBCL are uncommon and the disease remains poorly characterized. Our patient presented for management of osteomyelitis and was incidentally found to have a painless swelling and cyst around his right eye. A PET/CT scan revealed hypermetabolic activity within the lacrimal sac and a subsequent excisional biopsy of the mass yielded histopathology consistent with DLBCL. Consequently, the patient underwent treatment with R-CHOP therapy. The patient responded well to chemotherapy with a substantial shrinkage in tumor burden and the disease remained localized. Herein, we present a rare case of primary ocular lymphoma, highlight the importance of early diagnosis, and review current treatment modalities.

  14. Primary Diffuse Large B-Cell Lymphoma Localized to the Lacrimal Sac: A Case Presentation and Review of the Literature

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    Kevin Zarrabi

    2016-01-01

    Full Text Available We report a rare case of diffuse large B-cell lymphoma (DLBCL of the lacrimal sac in a 50-year-old male. The incidence of primary ocular lymphoma is low and it is considered a rare disease. Moreover, reports of ocular DLBCL are uncommon and the disease remains poorly characterized. Our patient presented for management of osteomyelitis and was incidentally found to have a painless swelling and cyst around his right eye. A PET/CT scan revealed hypermetabolic activity within the lacrimal sac and a subsequent excisional biopsy of the mass yielded histopathology consistent with DLBCL. Consequently, the patient underwent treatment with R-CHOP therapy. The patient responded well to chemotherapy with a substantial shrinkage in tumor burden and the disease remained localized. Herein, we present a rare case of primary ocular lymphoma, highlight the importance of early diagnosis, and review current treatment modalities.

  15. Four Lymphomas in 1 Patient: A Unique Case of Triple Composite Non-Hodgkin Lymphoma Followed by Classical Hodgkin Lymphoma.

    Science.gov (United States)

    Tennese, Alysa; Skrabek, Pamela J; Nasr, Michel R; Sekiguchi, Debora R; Morales, Carmen; Brown, Theresa C; Weisenburger, Dennis D; Perry, Anamarija M

    2017-05-01

    Composite lymphomas consist of 2 or more distinct lymphomas occurring in a single anatomical site or simultaneously in different sites and can be composed of any combination of B-cell non-Hodgkin lymphoma (NHL), T-cell NHL, or Hodgkin lymphoma (HL). Cases of composite lymphomas with more than 2 lymphomas are extremely rare, with only 4 reports in the literature. We report the case of a 49-year-old man with a triple composite lymphoma in a single lymph node, consisting of small lymphocytic lymphoma, follicular lymphoma, and mantle cell lymphoma in situ. The patient received multiple courses of chemotherapy and an autologous stem cell transplant, which resulted in complete remission. Then, 6 years after the stem cell transplant, he developed classical HL. This unique case is, to our knowledge, the first report of a patient with triple composite lymphoma consisting of 3 small mature B-cell NHLs, who subsequently developed a fourth lymphoma.

  16. Ultrasound presentation of abdominal non-Hodgkin lymphomas in pediatric patients.

    Science.gov (United States)

    Brodzisz, Agnieszka; Woźniak, Magdalena Maria; Dudkiewicz, Ewa; Grabowski, Dominik; Stefaniak, Jolanta; Wieczorek, Andrzej Paweł; Kowalczyk, Jerzy

    2013-12-01

    Burkitt's lymphoma accounts for approximately 25% of lymphomas diagnosed in children of developmental age. The tumor is localized mainly in the intestine (usually in the ileocecal region), mesenteric lymph nodes and extraperitoneal space. The clinical symptoms are non-specific and include: abdominal pain, vomiting, gastrointestinal bleeding, and acute abdomen suggesting appendicitis or intestinal intussusception. On ultrasound examination, Burkitt's lymphoma may manifest itself in various ways, depending on the origin of the lesion. The aim of this paper was to review the ultrasound manifestation of abdominal Burkitt's lymphoma in children. The analysis included 15 pediatric patients with Burkitt's non-Hodgkin lymphoma in the abdominal cavity. The mean age of the patients was 9.5. Abdominal and gastrointestinal ultrasound examinations were conducted using a Siemens scanner with a convex transducer of 3.5-5 MHz and linear array transducer of L4 - 7.5 MHz. Ultrasound examinations conducted in the group of 15 patients revealed pathological masses localized in the gastric wall in 3 patients (20%), in the ileocecal region in 10 patients (67%) and a disseminated process in 2 patients (13%). In 12 patients with a diagnosed Burkitt's non-Hodgkin lymphoma in an extragastric localization, differences in the morphology of the lesions were observed. The clinical and ultrasound picture of abdominal Burkitt's lymphoma in children is variable. A careful ultrasound assessment of all abdominal organs conducted with the use of convex and linear probes increases the chances of establishing an adequate diagnosis.

  17. Primary Diffuse Large B-Cell Lymphoma Localized to the Lacrimal Sac: A Case Presentation and Review of the Literature

    OpenAIRE

    Kevin Zarrabi; Ved Desai; Brandom Yim; Gabig, Theodore G.

    2016-01-01

    We report a rare case of diffuse large B-cell lymphoma (DLBCL) of the lacrimal sac in a 50-year-old male. The incidence of primary ocular lymphoma is low and it is considered a rare disease. Moreover, reports of ocular DLBCL are uncommon and the disease remains poorly characterized. Our patient presented for management of osteomyelitis and was incidentally found to have a painless swelling and cyst around his right eye. A PET/CT scan revealed hypermetabolic activity within the lacrimal sac an...

  18. Lenalidomide With or Without Rituximab in Treating Patients With Progressive or Relapsed Chronic Lymphocytic Leukemia, Small Lymphocytic Lymphoma, Prolymphocytic Leukemia, or Non-Hodgkin Lymphoma Previously Treated With Donor Stem Cell Transplant

    Science.gov (United States)

    2017-07-24

    Adult Nasal Type Extranodal NK/T-cell Lymphoma; Anaplastic Large Cell Lymphoma; Angioimmunoblastic T-cell Lymphoma; Cutaneous B-cell Non-Hodgkin Lymphoma; Extranodal Marginal Zone B-cell Lymphoma of Mucosa-associated Lymphoid Tissue; Hepatosplenic T-cell Lymphoma; Intraocular Lymphoma; Nodal Marginal Zone B-cell Lymphoma; Noncutaneous Extranodal Lymphoma; Peripheral T-cell Lymphoma; Prolymphocytic Leukemia; Recurrent Adult Burkitt Lymphoma; Recurrent Adult Diffuse Large Cell Lymphoma; Recurrent Adult Diffuse Mixed Cell Lymphoma; Recurrent Adult Diffuse Small Cleaved Cell Lymphoma; Recurrent Adult Grade III Lymphomatoid Granulomatosis; Recurrent Adult Immunoblastic Large Cell Lymphoma; Recurrent Adult Lymphoblastic Lymphoma; Recurrent Adult T-cell Leukemia/Lymphoma; Recurrent Cutaneous T-cell Non-Hodgkin Lymphoma; Recurrent Grade 1 Follicular Lymphoma; Recurrent Grade 2 Follicular Lymphoma; Recurrent Grade 3 Follicular Lymphoma; Recurrent Mantle Cell Lymphoma; Recurrent Marginal Zone Lymphoma; Recurrent Mycosis Fungoides/Sezary Syndrome; Recurrent Small Lymphocytic Lymphoma; Refractory Chronic Lymphocytic Leukemia; Refractory Hairy Cell Leukemia; Small Intestine Lymphoma; Splenic Marginal Zone Lymphoma; T-cell Large Granular Lymphocyte Leukemia; Testicular Lymphoma; Waldenström Macroglobulinemia

  19. Rituximab in Treating Patients Undergoing Donor Peripheral Blood Stem Cell Transplant for Relapsed or Refractory B-cell Lymphoma

    Science.gov (United States)

    2015-11-23

    B-cell Adult Acute Lymphoblastic Leukemia; B-cell Childhood Acute Lymphoblastic Leukemia; B-cell Chronic Lymphocytic Leukemia; Childhood Burkitt Lymphoma; Childhood Diffuse Large Cell Lymphoma; Childhood Immunoblastic Large Cell Lymphoma; Cutaneous B-cell Non-Hodgkin Lymphoma; Extranodal Marginal Zone B-cell Lymphoma of Mucosa-associated Lymphoid Tissue; Intraocular Lymphoma; Nodal Marginal Zone B-cell Lymphoma; Post-transplant Lymphoproliferative Disorder; Recurrent Adult Acute Lymphoblastic Leukemia; Recurrent Adult Burkitt Lymphoma; Recurrent Adult Diffuse Large Cell Lymphoma; Recurrent Adult Diffuse Mixed Cell Lymphoma; Recurrent Adult Diffuse Small Cleaved Cell Lymphoma; Recurrent Adult Grade III Lymphomatoid Granulomatosis; Recurrent Adult Hodgkin Lymphoma; Recurrent Adult Immunoblastic Large Cell Lymphoma; Recurrent Adult Lymphoblastic Lymphoma; Recurrent Childhood Acute Lymphoblastic Leukemia; Recurrent Childhood Grade III Lymphomatoid Granulomatosis; Recurrent Childhood Large Cell Lymphoma; Recurrent Childhood Lymphoblastic Lymphoma; Recurrent Childhood Small Noncleaved Cell Lymphoma; Recurrent Grade 1 Follicular Lymphoma; Recurrent Grade 2 Follicular Lymphoma; Recurrent Grade 3 Follicular Lymphoma; Recurrent Mantle Cell Lymphoma; Recurrent Marginal Zone Lymphoma; Recurrent Small Lymphocytic Lymphoma; Recurrent/Refractory Childhood Hodgkin Lymphoma; Refractory Chronic Lymphocytic Leukemia; Refractory Hairy Cell Leukemia; Small Intestine Lymphoma; Splenic Marginal Zone Lymphoma; Testicular Lymphoma; Waldenström Macroglobulinemia

  20. Paraneoplastic Syndrome in Splenic Marginal Zone Lymphoma: A Rare Phenomenon of Paraplegia as an Atypical Presenting Manifestation

    Directory of Open Access Journals (Sweden)

    Jessica Schering

    2016-01-01

    Full Text Available We describe a case presenting complaint of complete lower body paraparesis, which was discovered to have splenic marginal zone lymphoma (SMZL. While paraneoplastic syndromes are more common in tumors, such as small cell lung cancer, very few reports exist on this condition with SMZL. We describe such a rare entity with a clinical course spanning twenty-four months after diagnosis.

  1. Progressive multifocal cerebral infarction from intravascular large B cell lymphoma presenting in a man: a case report

    Directory of Open Access Journals (Sweden)

    Chotinaiwattarakul Wattanachai

    2011-01-01

    Full Text Available Abstract Introduction Intravascular lymphoma is rare, and may present as ischemic stroke. Diagnosis is difficult due to the non-specific presentation and lack of lymphadenopathy, thus leading to frequent instances of autopsy-proven diagnosis. To the best of our knowledge, this is the first report of progressive stroke from intravascular lymphoma diagnosed antemortem by random skin biopsy. Case presentation A 42-year-old Thai man presented to our hospital with progressive multifocal cerebral infarction. Despite taking aspirin (300 mg/day, his neurological symptoms worsened. During admission, he developed an unexplained fever and hypoxemia. Magnetic resonance angiography clearly showed patency of all cerebral arteries including the internal carotid and vertebrobasilar arteries. Echocardiography, an antiphospholipid antibody test, cerebrospinal fluid cytology and a bone marrow study were normal. Other laboratory test results showed an elevated lactate dehydrogenase level, nephrotic range proteinuria (3.91 g/day, hypoalbuminemia (1.9 g/dL, a very low high-density lipoprotein level (7 mg/dL and hypertriglyceridemia (353 mg/dL. Because of suspected vasculitis, pulse methylprednisolone was given with transiently minimal improvement. A random skin biopsy from both thighs revealed intravascular large B cell lymphoma. Chemotherapy was not given due to our patient having ventilator associated pneumonia. He died 10 days after the definite diagnosis was established. Conclusion One etiology of stroke is intravascular lymphoma, in which random skin biopsy can be helpful for antemortem diagnosis.

  2. Etoposide, Filgrastim, and Plerixafor in Improving Stem Cell Mobilization in Treating Patients With Non-Hodgkin Lymphoma

    Science.gov (United States)

    2016-09-15

    Adult Acute Lymphoblastic Leukemia in Remission; Adult Grade III Lymphomatoid Granulomatosis; Adult Nasal Type Extranodal NK/T-cell Lymphoma; Anaplastic Large Cell Lymphoma; Angioimmunoblastic T-cell Lymphoma; Cutaneous B-cell Non-Hodgkin Lymphoma; Extranodal Marginal Zone B-cell Lymphoma of Mucosa-associated Lymphoid Tissue; Hepatosplenic T-cell Lymphoma; Nodal Marginal Zone B-cell Lymphoma; Noncutaneous Extranodal Lymphoma; Peripheral T-cell Lymphoma; Recurrent Adult Burkitt Lymphoma; Recurrent Adult Diffuse Large Cell Lymphoma; Recurrent Adult Diffuse Mixed Cell Lymphoma; Recurrent Adult Diffuse Small Cleaved Cell Lymphoma; Recurrent Adult Grade III Lymphomatoid Granulomatosis; Recurrent Adult Immunoblastic Large Cell Lymphoma; Recurrent Adult Lymphoblastic Lymphoma; Recurrent Adult T-cell Leukemia/Lymphoma; Recurrent Cutaneous T-cell Non-Hodgkin Lymphoma; Recurrent Grade 1 Follicular Lymphoma; Recurrent Grade 2 Follicular Lymphoma; Recurrent Grade 3 Follicular Lymphoma; Recurrent Mantle Cell Lymphoma; Recurrent Marginal Zone Lymphoma; Recurrent Mycosis Fungoides/Sezary Syndrome; Recurrent Small Lymphocytic Lymphoma; Refractory Chronic Lymphocytic Leukemia; Refractory Hairy Cell Leukemia; Small Intestine Lymphoma; Splenic Marginal Zone Lymphoma; T-cell Large Granular Lymphocyte Leukemia; Testicular Lymphoma; Waldenström Macroglobulinemia

  3. Durable survival after chemotherapy in a HIV patient with Burkitt's lymphoma presenting with massive upper gastrointestinal bleeding.

    Science.gov (United States)

    Law, Man F; Chan, Hay N; Pang, Chun Y; Lai, Ho K; Ha, Chung Y; Ng, Celia; Ho, Rita; Wong, Cheuk K; Yeung, Yiu M; Yip, Sze F

    2016-07-01

    Massive upper gastrointestinal bleeding is an uncommon presentation of Burkitt's lymphoma in a patient with HIV/AIDS, and is seldom reported in the literature. A 39-year-old man who has sex with men presented with abdominal pain and massive haematemesis and a rapid drop in haemoglobin level to 4.8 g/dL. Upper gastrointestinal endoscopy showed a large blood clot in the stomach, and an emergency laparotomy was performed because of unstable haemodynamics. This showed active bleeding from multiple tumours in the fundus and body of the stomach. The patient underwent gastrectomy and gastric biopsy confirmed Burkitt's lymphoma. Further tests showed lymphoma involvement in bone marrow and central nervous system. The patient tested positive for HIV, and had a CD4 count of 212 cells/mm(3) and viral load of 18,000 copies/mL at diagnosis. He was commenced on a chemotherapy regimen of CODOX-M/IVAC, and highly active antiretroviral therapy consisting of indinavir, stavudine and lamivudine. The major side effect was peripheral neuropathy. Infective complications during chemotherapy were controlled by broad-spectrum antibiotics and anti-fungal agents. Complete remission of the lymphoma was achieved after the chemotherapy and remission was maintained for more than 14 years. © The Author(s) 2015.

  4. Ultrasound presentation of abdominal non-Hodgkin lymphomas in pediatric patients

    Directory of Open Access Journals (Sweden)

    Agnieszka Brodzisz

    2013-12-01

    Full Text Available Introduction: Burkitt’s lymphoma accounts for approximately 25% of lymphomas diagnosed in children of developmental age. The tumor is localized mainly in the intestine (usually in the ileocecal region, mesenteric lymph nodes and extraperitoneal space. The clinical symptoms are non-specific and include: abdominal pain, vomiting, gastrointestinal bleeding, and acute abdomen suggesting appendicitis or intestinal intussusception. On ultrasound examination, Burkitt’s lymphoma may manifest itself in various ways, depending on the origin of the lesion. Aim: The aim of this paper was to review the ultrasound manifestation of abdominal Burkitt’s lymphoma in children. Material and methods: The analysis included 15 pediatric patients with Burkitt’s non-Hodgkin lymphoma in the abdominal cavity. The mean age of the patients was 9.5. Abdominal and gastrointestinal ultrasound examinations were conducted using a Siemens scanner with a convex transducer of 3.5–5 MHz and linear array transducer of L4 – 7.5 MHz. Results: Ultrasound examinations conducted in the group of 15 patients revealed pathological masses localized in the gastric wall in 3 patients (20%, in the ileocecal region in 10 patients (67% and a disseminated process in 2 patients (13%. In 12 patients with a diagnosed Burkitt’s non-Hodgkin lymphoma in an extragastric localization, differences in the morphology of the lesions were observed. Conclusions: The clinical and ultrasound picture of abdominal Burkitt’s lymphoma in children is variable. A careful ultrasound assessment of all abdominal organs conducted with the use of convex and linear probes increases the chances of establishing an adequate diagnosis.

  5. Fulminate hepatic failure as an initial presentation of non-hodgkin lymphoma: a case report.

    Science.gov (United States)

    Ahmadi, Bizhan; Shafieipour, Sara; Akhavan Rezayat, Kambiz

    2014-04-01

    Viral hepatitis and toxins comprise most common causes of fulminate hepatic failure that are often diagnosed with standard laboratory tests. Herein we discuss a rare, difficult to diagnosis etiology of acute liver failure (ALF). A 62-year-old man presented with a two-week history of fever and fatigue. At four days before admission he became lethargic. His past medical and drug histories were unremarkable. Physical examination revealed generalized jaundice, fever and loss of consciousness. Laboratory tests showed elevated liver transaminases with direct hyper-bilirubinemia. Abdominal ultrasonography and CT scan showed hepatosplenomegaly and para-aortic abdominal lymphadenopathy. A further work-up included liver biopsy. The histopathology and imunohistochemistry was compatible with diffuse large B-cell lymphoma. He underwent high dose glucocorticoid therapy but his condition deteriorated rapidly and he died eight days after admission. ALF as an initial manifestation of malignant hepatic infiltration is extremely rare yet should be considered in all patients with unknown hepatic failure that are highly suspicious for malignant neoplasm.

  6. ALK-positive anaplastic large cell lymphoma presenting as intradural spinal mass: first reported case and review of literature.

    Science.gov (United States)

    Novello, Mariangela; Lauriola, Libero; Della Pepa, Giuseppe Maria; Giuseppe, La Rocca; Coli, Antonella; Visocchi, Massimiliano

    2013-08-01

    Anaplastic large cell lymphoma (ALCL) is characterized by large anaplastic cells of T-cell or null-cell phenotype expressing CD30 (Ki-1 antigen). In most cases this neoplasm expresses the anaplastic lymphoma kinase (ALK), a chimeric protein resulting from the t(2;5)(p23;q35) translocation. ALK-positive anaplastic large cell lymphoma is most frequent in the first three decades of life and shows a male predominance, involving both nodal and extranodal sites, but rarely the CNS. We report a 21-year-old patient with a previous history of nodal ALK-positive ALCL, lymphohistiocytic subtype, who was admitted for recent occurrence of left-sided anesthesia with pain and progressive motor weakness of both legs. An MRI of the spine documented an intradural extramedullary mass dislocating the thoracic cord, suggesting a meningioma and the patient underwent surgical decompression. Histological examination revealed a lymphoproliferative neoplasm with morphology and immunophenotype of ALK-positive anaplastic large cell lymphoma. After surgery, all preoperative symptoms disappeared. To our knowledge, no cases of ALCL presenting as secondary localization with an intradural extramedullary spinal mass have been reported in the literature. © 2012 Japanese Society of Neuropathology.

  7. Various Neurological Symptoms by Neurolymphomatosis as the Initial Presentation of Primary Testicular Lymphoma

    Directory of Open Access Journals (Sweden)

    Yoshitaka Sunami

    2015-04-01

    Full Text Available Neurological symptoms induced by the infiltration of malignant lymphoma into the nervous systems are subsumed under the term neurolymphomatosis (NL. Here, we report the case of a 30-year-old Japanese man with primary testicular lymphoma complicated, as seen in various neurological findings, by secondary NL prior to testicular swelling. Painless right scrotal enlargement was noticed more than 1 month after the appearance of neurological complications such as right upper extremity numbness, dysarthria, facial palsy, and diplopia. Proactive investigation and biopsies of extranodal sites at high risk of central nervous system infiltration of malignant lymphoma, such as the testes, should be considered when secondary NL is suspected based on imaging findings.

  8. B-cell and T-cell lymphomas of the breast: clinical--pathological features of 53 cases.

    Science.gov (United States)

    Gualco, Gabriela; Bacchi, Carlos E

    2008-10-01

    Breast involvement by non-Hodgkin lymphomas is rare. We studied the morphological, immunophenotypical, and clinical features of 53 cases of malignant lymphomas involving the breast in a population of Brazilian patients. Most of the cases were of B-cell phenotype. Four of the patients with primary breast lymphomas had T-cell lymphomas, 3 had CD30-positive anaplastic large cell lymphomas, and 1 had panniculitis-like T-cell lymphoma. Most patients presented with an incidental breast mass. Secondary breast lymphoma was seen in 19 patients and most commonly occurred as part of widespread nodal disease. Two patients presented with bilateral breast involvement. The most prevalent histological subtype was also diffuse large B-cell lymphoma, followed by follicular lymphoma. This study shows that the broad morphological and immunophenotypical spectrum of malignant lymphoma of the breast occurring in a large series of Brazilian patients has many similarities with that seen in Western countries, with a higher proportion of high-grade lymphomas in both primary and secondary cases.

  9. Non-Hodgkin's lymphomas; Lymphomes malins non hodgkiniens

    Energy Technology Data Exchange (ETDEWEB)

    Drouet, F.; Mahe, M.A. [Service de radiotherapie du centre Rene-Gauducheau, CRLCC Nantes-Atlantique, 44 - Saint-Herblain (France); Cahu, X. [Service d' hematologie clinique CHU de Rennes, hopital Pontchaillou, 35 - Rennes (France); Pointreau, Y. [Service de radiotherapie, centre regional universitaire de cancerologie Henry-S.-Kaplan CHU de Tours, Hpital Bretonneau, 37 - Tours (France); Denis, F. [Centre Jean-Bernard, Service de radiotherapie 72 - Le Mans (France)

    2010-07-01

    With approximately 10000 cases per year in France, non-Hodgkin's lymphoma (NHL) represents the most frequent hematological malignancy, and 5 to 10 % of new cases of cancers. NHLs constitute a heterogeneous group of lympho-proliferative diseases, including entities with very different epidemiological and evolutive characteristics, as well as prognosis and treatments. Several classifications exist, but in practice, we individualize aggressive NHL including Diffuse Large B-Cell Lymphomas (DLBCL) which is the most common lymphoma, and indolent NHL including follicular lymphomas and mucosa-associated lymphoid tissue (MALT) lymphomas. The role of the radiotherapy in the management of NHLs varies according to the specific sub-type of lymphoma, but it has become increasingly limited over time. Overall it finds indications with curative intent only in situations of localized LMNH: either associated with chemotherapy as part of a combined modality therapy as for the treatment of localized DLBCL, or as exclusive treatment specially in the rare situations of localized follicular lymphomas. Moreover, lymphocytes being extremely radiosensitive cells, radiotherapy retains excellent indications with palliative intent for the management of symptomatic bulky tumor masses, and that whatever the sub-type of NHLs may be. It is important to remember that even today the 'Involved Field' irradiation type remains the gold standard for the treatment of nodal NHLs, even if we witness at present the emergence of new types of irradiation, which aim to reduce the amount of irradiated tissues to try to limit the risks of delayed radio-induced complications. The purpose of this article is to clarify the specific aspects (epidemiological, radio-anatomical and prognostic characteristics) of each NHLs'sub-types (except primary central nervous system lymphomas), as well as the practical modalities of the irradiation (illustrated by a clinical case record) when an indication of

  10. DISTURBED ANTIGEN PRESENTATION IN CLASSICAL HODGKIN LYMPHOMA; IMPLICATIONS FOR IMMUNE CHECKPOINT INHIBITOR THERAPY?

    NARCIS (Netherlands)

    Nijland, M.; Visser, Lydia; Veenstra, Rianne; Kushekhar, K.; van Imhoff, G.; Berg, van den Anke; Diepstra, A.

    2016-01-01

    Immune checkpoint inhibitors are being tested in clinical trials and show great promise in the treatment of classical Hodgkin lymphoma (cHL). The proposed mechanism of action of these inhibitors consists of reactivating T lymphocytes that have become unresponsive as a consequence of inhibitory mecha

  11. Treatment of follicular NHL: The old and the new

    Science.gov (United States)

    Friedberg, Jonathan W.

    2008-01-01

    Despite remaining an incurable disease, overall survival improvements have been noted in patients with advanced stage follicular lymphoma. The Follicular Lymphoma International Prognostic Index (FLIPI) is a robust prognostic index in this disease, and continues to provide prognostic information in the rituximab era. Rituximab has significantly changed the management of follicular lymphoma, and the most dramatic impact of rituximab is observed in combination with cytotoxic chemotherapy. However, resistance to rituximab remains a problem, and standard therapy in the rituximab-refractory setting includes radioimmunotherapy, autologous stem cell transplantation, and allogeneic stem cell transplantation. In addition, several novel agents show encouraging activity in FL, including bendamustine, lenalidomide, bortezomib and other proteasome inhibitors, and BCL2 inhibitors. PMID:18760706

  12. Canine small clear cell/T-zone lymphoma: clinical presentation and outcome in a retrospective case series.

    Science.gov (United States)

    Martini, V; Marconato, L; Poggi, A; Riondato, F; Aresu, L; Cozzi, M; Comazzi, S

    2016-08-01

    Published studies, taken together, suggest the existence of a single canine lymphoma entity, with a small clear cell appearance by cytological evaluation, a histopathological T-zone pattern and an aberrant CD45-negative T-cell phenotype, mostly characterized by long-term survival. We describe clinical presentation and outcome in a retrospective case series of canine small clear cell/T-zone lymphoma. Despite the reported predisposition of Golden retriever, this breed was not represented in our case series. Most dogs presented with stage V disease, whereas only few had clinical signs or peripheral cytopenias. Blood was almost always more infiltrated than bone marrow. Median survival confirmed the favourable prognosis described in literature, but a few dogs died within a short time. Also, a subgroup of dogs developed second malignancies, eventually leading to death. We did not investigate possible prognostic factors because of the wide variety in treatments, and further studies are needed to identify high-risk animals.

  13. Extranodal natural killer cell/t-cell lymphoma, nasal type, presenting as cutaneous nodules and a small-bowel perforation.

    Science.gov (United States)

    Agarwal, Priyanka; Ruzinova, Marianna B; Harris, Marian H; Qureshi, Abrar A; Stebbins, William G

    2010-02-01

    Extranodal natural killer (NK) cell/T-cell lymphoma, nasal type, is a rare aggressive neoplasm, most commonly presenting as a destructive lesion in the nasal cavity and nasopharynx in middle-aged to older adults. About one third of cases present in an extranasal location, commonly involving skin and gastrointestinal tract, and usually occur in the absence of superficial lymphadenopathy. Diagnosis of this malignancy can be missed given its rarity and heterogeneous presentation. We describe a patient with an extranodal NK cell/T-cell lymphoma, nasal type, who was initially diagnosed and treated for a presumed Mycobacterium marinum infection, after biopsies were unrevealing. However, after more serious complications developed, repeat biopsy was performed. An atypical lymphocytic infiltrate was noted, with cells being positive for NK cell/T-cell markers CD2, CD7, and CD3 (subset), as well as for cytotoxic lymphocyte markers perforin, T-cell intracellular antigen, and CD56. In situ hybridization for Epstein-Barr virus-encoded RNA was also positive. This case demonstrates an important diagnostic pitfall of confusing cutaneous involvement by an aggressive NK cell/T-cell lymphoma with an antibiotic-resistant infection. Repeat biopsies and close clinicopathologic correlation are essential for establishment of correct diagnosis.

  14. Hypercalcemia and huge splenomegaly presenting in an elderly patient with B-cell non-Hodgkin's lymphoma: a case report

    Directory of Open Access Journals (Sweden)

    Tirgari Farrokh

    2010-10-01

    Full Text Available Abstract Introduction Hypercalcemia is the major electrolyte abnormality in patients with malignant tumors. It can be due to localized osteolytic hypercalcemia or elaboration of humoral substances such as parathyroid hormone-related protein from tumoral cells. In hematological malignancies, a third mechanism of uncontrolled synthesis and secretion of 1-25(OH2D3 from tumoral cells or neighboring macrophages may contribute to the problem. However, hypercalcemia is quite unusual in patients with B-cell non-Hodgkin's lymphoma. Case presentation An 85-year-old Caucasian woman presented with low grade fever, anorexia, abdominal discomfort and fullness in her left abdomen for the last six months. She was mildly anemic and complained of fatigability. She had huge splenomegaly and was hypercalcemic. After correction of her hypercalcemia, she had a splenectomy. Microscopic evaluation revealed a malignant lymphoma. Her immunohistochemistry was positive for leukocyte common antigen, CD20 and parathyroid hormone-related peptide. Conclusion Immunopositivity for parathyroid hormone-related peptide clearly demonstrates that hypersecretion of a parathyroid hormone-like substance from the tumor had led to hypercalcemia in this case. High serum calcium is seen in only seven to eight percent of patients with B-cell non-Hodgkin's lymphoma, apparently due to different mechanisms. Evaluation of serum parathyroid hormone-related protein and 1-25(OH2D3 can be helpful in diagnosis and management. It should be noted that presentation with hypercalcemia has a serious impact on prognosis and survival.

  15. Hodgkin's Lymphoma

    Science.gov (United States)

    ... behavior. Your type determines your treatment options. Classical Hodgkin's lymphoma Classical Hodgkin's lymphoma is the more common ... Hodgkin's lymphoma Lymphocyte-rich Hodgkin's lymphoma Lymphocyte-predominant Hodgkin's lymphoma This much rarer type of Hodgkin's lymphoma ...

  16. Non-Hodgkin lymphoma presenting as bilateral tonsillar hypertrophy: case report.

    LENUS (Irish Health Repository)

    Khan, Sardar U

    2012-02-01

    We describe the case of a 57-year-old man who was referred to us with persistent sore throat, dysphagia, and enlarged tonsils. He had not responded to earlier treatment with antibiotic therapy and other routine measures. In view of the persistent nature of the patient\\'s symptoms and the tonsillar hypertrophy, we decided to perform a tonsillectomy and to send the excised specimens for pathologic analysis. Histologic evaluation identified non-Hodgkin lymphoma in both tonsils. The patient was treated with postoperative chemo- and radiotherapy, and he was free of symptoms during 18 months of follow-up. To the best of our knowledge, only 4 cases of bilateral non-Hodgkin lymphoma of the tonsils have been reported in the English-language literature. We also discuss the importance of histologic analysis of excised tonsil tissue in selected cases.

  17. A Patient with Supraclavicular Lymphadenopathy and Anterior Mediastinal Mass Presenting as a Rare Case of Composite Lymphoma: A Case Report and Literature Review

    Directory of Open Access Journals (Sweden)

    Alex Raufi

    2016-12-01

    Full Text Available Composite lymphoma (CL is a rare disease with 2 distinct lymphomas concurrently arising in a single patient with an estimated incidence of 1–4.7% of newly diagnosed lymphomas per year. CL most commonly involves 2 B-cell non-Hodgkin lymphomas (NHL or a B-cell NHL with a Hodgkin lymphoma. Our case is unique in that it was a bilineage CL with both a T-cell and B-cell NHL, which has only been reported in a few case reports. A 49-year-old woman presented with several months of progressive cough, weight loss, dyspnea, and supraclavicular lymphadenopathy. Computed tomographic imaging done upon admission to the hospital found that she had extensive anterior and middle mediastinal lymphadenopathy as well as bilateral supraclavicular lymphadenopathy. The patient underwent an excisional biopsy on the supraclavicular lymph node and was found to have a composite lymphoma involving both a T-cell and B-cell NHL. Her final pathological diagnosis was peripheral T-cell lymphoma and lymphoplasmacytic lymphoma. The patient was found to have stage IIIB disease. Her HIV, hepatitis panel, and tuberculosis tests were all negative. She then underwent chemotherapy with dose-adjusted EPOCH-R (etoposide, prednisone, vincristine, cyclophosphamide, doxorubicin, and rituximab. The patient showed a complete response and was then referred to a bone marrow transplant center for an autologous hematopoietic stem cell transplant. CL is a rare disease composed of at least 2 distinct lymphomas concurrently arising in a single patient. Due to the complexity in having to treat multiple types of lymphoma simultaneously CL presents challenges with treatment and assessing prognosis.

  18. Peripheral T-cell lymphoma presenting as an ischemic stroke in a 23-year-old woman: a case report and review of the literature

    Directory of Open Access Journals (Sweden)

    Fragou Mariantina

    2009-10-01

    Full Text Available Abstract Introduction Peripheral T-cell lymphoma of the unspecified variant is a highly aggressive subtype of T-cell non-Hodgkin's lymphoma. This is the first reported case of this type of lymphoma presenting as an ischemic stroke in a woman. Case presentation A previously healthy 23-year-old woman presented with fever and hemiplegia. She was subsequently intubated after scoring 7 out of 15 at the Glasgow Coma Scale. Brain computed tomography scans of the patient depicted a massive sylvian infarction while an abdominal computed tomography scan revealed multiple enlarged abdominal lymph nodes and a retroperitoneal mass adjacent to the left psoas muscle. A diagnostic work up for inherited thrombophilia yielded negative results. Blood and cerebrospinal fluid cultures for infectious agents also gave negative results. A biopsy of the retroperitoneal mass guided by computed tomography was inconclusive. A biopsy of an enlarged inguinal lymph node of the patient, combined with an immunophenotypic analysis, revealed an unspecified variant of peripheral T-cell lymphoma. The patient underwent chemotherapy but developed multiple organ failure. She died 26 days after she was admitted to our intensive care unit. Conclusion Peripheral T-cell lymphoma of the unspecified variant is a highly aggressive subtype of peripheral T-cell lymphomas. The latter exhibit no consistent immunophenotypic, genetic, or clinical features. Clinicians should be aware of atypical clinical presentations of the above lymphomas such as ischemic stroke.

  19. Refractory Classical Hodgkin Lymphoma Presenting with Atypical Cutaneous Involvement and Diagnosis of ZZ Phenotype Alpha-1 Antitrypsin Deficiency

    Directory of Open Access Journals (Sweden)

    Mohamad Khawandanah

    2014-01-01

    Full Text Available Cutaneous Hodgkin lymphoma is a rare condition. Specific neoplastic involvement can be primary (confined to the skin or secondary to systemic involvement (metastatic. Cutaneous involvement by HL usually occurs late in the course and is associated with poor prognosis; however in some cases it can exhibit indolent behavior. Skin involvement with nonspecific cutaneous findings may represent a paraneoplastic syndrome. We describe a case of 46-year-old white male patient presented with rash and lymphadenopathy which led to the diagnosis of stage IVE mixed cellularity classical Hodgkin lymphoma with skin involvement. His disease was refractory to multiple lines of chemotherapy including (1 AVD (doxorubicin/bleomycin/dacarbazine, (2 brentuximab, and (3 bendamustine, he later achieved complete remission with (4 GCD (gemcitabine/carboplatin/dexamethasone salvage regimen. Bleomycin was not given secondary to poor pulmonary function tests. His treatment was complicated after AVD with multiple pneumothoraces which unmasked the diagnosis of ZZ phenotype alpha-1 antitrypsin (ATT deficiency. Simultaneous existence of Hodgkin lymphoma and ATT is rarely reported.

  20. An Unusual Case of Plasmablastic Lymphoma Presenting as Paravertebral Mass Evaluated by {sup 18}F-FDG PET/CT

    Energy Technology Data Exchange (ETDEWEB)

    Treglia, Giorgio; Paone, Gaetano; Stathis, Anastasios; Ceriani, Luca; Giovanella, Luca [Oncology Institute of Southern Switzerland, Bellinzona (Switzerland)

    2014-03-15

    A 60-year-old man underwent radiological investigations due to the onset of back pain. Computed tomography (CT) and magnetic resonance imaging (MRI) showed the presence of a paravertebral mass located ahead the body of the third thoracic vertebra. Based on these findings the patient underwent biopsy of the paravertebral mass, which showed the presence of a plasmablastic lymphoma. Therefore, the patient underwent fluorine-18-fluorodeoxyglucose positron emission tomography/computed tomography ({sup 18}F-FDG PET/CT) for staging. Before {sup 18}F-FDG injection, the patient had fasted for at least 6 h; at the time of the radiopharmaceutical injection he presented glucose blood levels corresponding to 98 mg/dl. Images were acquired 1 h after intravenous injection of 280 MBq of {sup 18}F-FDG according to the body mass index. PET images were interpreted visually and semiquantitatively by using the maximal standardized uptake value (SUVmax). {sup 18}F-FDG PET/CT showed moderate radiopharmaceutical uptake corresponding to the paravertebral lesion (SUVmax 3.3) and diffuse uptake in the skeleton suspicious for bone marrow neoplastic involvement, with more evident hypermetabolic areas in the left scapula (SUVmax 3.7), right sixth rib (SUVmax 3.5), and left iliac bone (SUVmax 3.4) (Fig. 1). Subsequent bone marrow biopsy confirmed the bone marrow infiltration by plasmablastic cells. Based on these findings, a final diagnosis of plasmablastic lymphoma with bone marrow involvement was performed and the patient was addressed to chemotherapy. Plasmablastic lymphoma is a rare CD20-negative large-cell lymphoma with plasmablastic features occurring primarily in HIV or Epstein-Barr virus positive individuals. Distinguishing this tumor from myeloma could be challenging. The most frequent site of presentation is the oral cavity, whereas extraoral localizations of plasmablastic lymphoma are considered to be very rare and they should be differentiated from extraosseous localization of

  1. Análisis molecular de la longitud telomérica en linfomas foliculares: Su participación en la progresión tumoral Molecular analysis of telomere length in follicular lymphomas. Its participation in tumor progression

    Directory of Open Access Journals (Sweden)

    Alejandra S. H. Cottliar

    2005-04-01

    Full Text Available Los telómeros son estructuras esenciales para el mantenimiento de la integridad cromosómica y la capacidad replicativa de la célula. La reducción de la longitud telomérica (LT aumenta la probabilidad de producir errores capaces de generar cambios genómicos importantes para el desarrollo neoplásico, determinando desbalances de material genético. En este trabajo se evaluó la LT mediante el análisis de fragmentos de restricción terminal (TRF en médula ósea y/o biopsia ganglionar de 36 pacientes (edad media: 54.2 años; rango 29-77 años; 21 varones: 29 con linfoma folicular (LF al diagnóstico y 7 con linfoma B difuso a células grandes secundario a LF (LBDCG-S. Se efectuó el análisis del rearreglo molecular del gen BCL-2 por PCR anidada y de larga distancia. Las medias de TRF en LF (4.18±0.18 Kb y LBDCG-S (3.31±0.25 Kb resultaron significativamente menores que en controles (8.50±0.50 Kb (pTelomeres are essential for maintaining chromosomal integrity and stability. We studied here telomere length (TL in bone marrow and/or lymph node from 36 patients: 29 with follicular lymphoma (FL at diagnosis and 7 with diffuse large B cell lymphoma secondary to FL (S-DLBCL. TL was evaluated using terminal restriction fragments (TRF assay. BCL-2 gene rearrangement was analyzed by nested and long distance PCR. Mean TRF values showed significant telomere shortening in FL (4.18±0.18 Kb and S-DLBCL (3.31±0.25 Kb respect to controls (8.50±0.50 Kb (p<0.001. Differences between both histological subtypes (p=0.036 were also detected. Moreover, the samples positive for BCL-2 rearrangements showed longer TL (4.25±0.19 Kb than the negative ones (3.39±0.30 Kb (p=0.023. A trend to telomere shortening was observed when Major Breakpoint Region (MBR-J H, minor cluster region (mcr-J H and BCL-2 negative patients were compared (4.35±0.21 Kb; 3.84±0.45 Kb and 3.39±0.30 Kb, respectively. Our results show a TL reduction in FL and S-DLBCL, with significant

  2. Unusual case of Hodgkin lymphoma presenting as soft tissue mass with intracranial metastasis and review of literature

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    Vandana Singh Kushwaha

    2015-01-01

    Full Text Available Hodgkin lymphoma (HL primarily presents as nodal disease and may involve extranodal sites during the progression of the disease. Extra nodal involvement of lung, gastrointestinal tract, testis and thyroid etc., is well-recognized in non-HL but clinically detectable soft tissue involvement is rare and quite exceptional with HL. We report here an unusual case of a young adult male, who presented with a frontal soft tissue mass associated with cervical lymph node. Histopathology supplemented with immunohistochemistry revealed classical HL. Patient did not respond to treatment adequately, and he developed intracranial metastasis during the course of treatment.

  3. Evans Syndrome Presented with Marginal Zone Lymphoma and Duodenal Neuroendocrine Tumor in an Elderly Woman

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    Daniele D'Ambrosio

    2016-12-01

    Full Text Available Evans syndrome (ES is an autoimmune disorder characterized by simultaneous or sequential development of autoimmune hemolytic anemia, immune thrombocytopenia, and/or neutropenia. ES can be classified as a primary (idiopathic or secondary (associated with an underlying disease syndrome. We report a case of ES in an elderly patient in the presence of multiple trigger factors such as recent influenza vaccine, marginal zone lymphoma, and neuroendocrine tumor G1. Whether this association is casual or causal remains a matter of speculation. It is however necessary to have a thorough work-up in a newly diagnosed ES and a more accurate search of miscellaneous factors especially in elderly patients.

  4. Angiolymphoid hyperplasia with follicular mucinosis

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    Joshi Rajiv

    2007-01-01

    Full Text Available Follicular mucinosis occurring along with angiolymphoid hyperplasia with eosinophils (ALHE has been described in a 54-year-old female. The patient presented with pruritic erythematous papules on the left frontoparietal scalp. Histopathological examination showed prominent blood vessels in the dermis lined by plump histiocytoid endothelial cells that were surrounded by a dense lymphoid infiltrate with numerous eosinophils; these findings are typical of angiolymphoid hyperplasia with eosinophils. Features of follicular mucinosis were observed in the same section with several hyperplastic follicular infundibula containing pools of mucin in the infundibular epithelium. The concurrent occurrence of these two distinct histopathological patterns in the same biopsy specimen has been described in only three cases to date.

  5. Gastric lymphoma

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    Sravani Padala

    2016-06-01

    Full Text Available Gastrointestinal lymphomas represent 5-20% of extra nodal lymphomas and mainly occur in the stomach and small intestine. Clinical findings are not specific, thus often determining a delay in the diagnosis. Imaging features at conventional and cross-sectional imaging must be known by the radiologist since he/she plays a pivotal role in the diagnosis and disease assessment, thus assisting in the choice of the optimal treatment to patients. This review focuses on the wide variety of imaging presentation of esophageal, gastric, and small and large bowel lymphoma presenting their main imaging appearances at conventional and cross-sectional imaging, mainly focusing on computed tomography and magnetic resonance, helping in the choice of the best imaging technique for the disease characterization and assessment and the recognition of potential complications. Gastrointestinal tract is the most common extra nodal site involved by lymphoma. Although lymphoma can involve any part of the gastrointestinal tract .The most frequent sites in order of its occurrence are the stomach followed by small intestine and ileocecal region. Gastrointestinal tract lymphoma is usually secondary to the widespread nodal diseases and primary gastrointestinal tract lymphoma is relatively rare. [Int J Res Med Sci 2016; 4(6.000: 2481-2486

  6. New modalities (setting, fractionation) of radioimmunotherapy by {sup 90}Y-ibritumomab tiuxetan ({sup 90}Y zevalin) in first line treatment of follicular type non Hodgkin malignant lymphomas: efficiency, toxicity and personalized dosimetry approach; Nouvelles modalites (consolidation, fractionnement) de radioimmunotherapie par {sup 90}Y-ibritumomab tiuxetan (Zevalin) en traitement de premiere ligne des lymphomes malin non hodgkiniens de type folliculaire: efficacite, toxicite et approche dosimetrique personnalisee

    Energy Technology Data Exchange (ETDEWEB)

    Morschhauser, F

    2008-12-15

    Rationale: radioimmunotherapy (R.I.T.) with {sup 90}Y-ibritumomab tiuxetan ([{sup 90}Y] Zevalin ) is a new treatment option for patients with relapsed/refractory non Hodgkin follicular lymphoma (F.L.). Efficacy increases when Zevalin is used earlier in the disease course. Currently, Zevalin dosage is based on weight and not dosimetry. This most likely results in a wide range of absorbed dose to critical organs and tumor, which in turn translates in unpredictable efficacy and toxicity. Optimizing R.I.T. with [{sup 90}Y] Zevalin will require its use as part of first-line therapy and implementation of patient-specific dosimetry methods in clinical trials. Objectives and methods: we have consecutively studied 2 new modalities of using Zevalin in first line therapy of F.L.. First, we conducted an international, randomized, phase 3 trial to evaluate the efficacy and safety of consolidation with Zevalin(15 MBq/Kg) in patients with advanced-stage F.L. achieving at least a partial response after induction immuno chemotherapy. A second approach consisted of evaluating a fractionated schedule with 2 doses of Zevalin (11.1 MBq/kg each), 9 to 13 weeks apart, as front line therapy in F.L. patients with high tumor burden. As part of this second approach, we designed a refined imaging-based (planar and 3-dimensional) dosimetry protocol to improve prediction of dose efficacy and toxicity after each dose of zevalin. Data acquisition was performed in 3 centers (Lille, Nantes and Manchester) while data treatment and specific dose calculations for major organ, tumor masses and bone marrow were centralized. Conclusion: Consolidation of first remission with {sup 90}Y-ibritumomab tiuxetan in advanced-stage follicular lymphoma is highly effective with no unexpected toxicities, prolonging P.F.S. by 2 years and resulting in high P.R.-to-C.R. conversion rates regardless of type of first-line induction treatment. Preliminary data show the feasibility of front line fractionated R.I.T. with

  7. Low-Dose Total Body Irradiation and Donor Peripheral Blood Stem Cell Transplant Followed by Donor Lymphocyte Infusion in Treating Patients With Non-Hodgkin Lymphoma, Chronic Lymphocytic Leukemia, or Multiple Myeloma

    Science.gov (United States)

    2015-10-30

    Adult Nasal Type Extranodal NK/T-cell Lymphoma; Anaplastic Large Cell Lymphoma; Angioimmunoblastic T-cell Lymphoma; Cutaneous B-cell Non-Hodgkin Lymphoma; Extranodal Marginal Zone B-cell Lymphoma of Mucosa-associated Lymphoid Tissue; Hepatosplenic T-cell Lymphoma; Intraocular Lymphoma; Nodal Marginal Zone B-cell Lymphoma; Noncutaneous Extranodal Lymphoma; Peripheral T-cell Lymphoma; Recurrent Adult Acute Lymphoblastic Leukemia; Recurrent Adult Burkitt Lymphoma; Recurrent Adult Diffuse Large Cell Lymphoma; Recurrent Adult Diffuse Mixed Cell Lymphoma; Recurrent Adult Diffuse Small Cleaved Cell Lymphoma; Recurrent Adult Grade III Lymphomatoid Granulomatosis; Recurrent Adult Immunoblastic Large Cell Lymphoma; Recurrent Adult Lymphoblastic Lymphoma; Recurrent Adult T-cell Leukemia/Lymphoma; Recurrent Cutaneous T-cell Non-Hodgkin Lymphoma; Recurrent Grade 1 Follicular Lymphoma; Recurrent Grade 2 Follicular Lymphoma; Recurrent Grade 3 Follicular Lymphoma; Recurrent Mantle Cell Lymphoma; Recurrent Marginal Zone Lymphoma; Recurrent Mycosis Fungoides/Sezary Syndrome; Recurrent Small Lymphocytic Lymphoma; Refractory Chronic Lymphocytic Leukemia; Refractory Hairy Cell Leukemia; Refractory Multiple Myeloma; Small Intestine Lymphoma; Splenic Marginal Zone Lymphoma; Stage II Multiple Myeloma; Stage III Multiple Myeloma; Testicular Lymphoma; Waldenström Macroglobulinemia

  8. Pseudomembranous colitis presenting as acute colonic obstruction without diarrhea in a patient with gastric Burkitt lymphoma

    Institute of Scientific and Technical Information of China (English)

    Kenichi Nomura; Shigeo Horiike; Takeshi Okanoue; Masafumi Taniwaki; Kohei Fukumoto; Daisuke Shimizu; Takashi Okuda; Naohisa Yoshida; Yuri Kamitsuji; Yosuke Matsumoto; Hideyuki Konishi; Yuji Ueda

    2005-01-01

    Pseudomembranous colitis (PMC) usually manifests asfever and diarrhea in hospitalized patients treated withsystemic antibiotics. We described a case of PMC withintestinal obstruction but without diarrhea. A 60-yearold man was hospitalized for chemotherapy for the treatment of Burkitt lymphoma of the stomach. The patient became febrile and complained of crampy abdominal pain during the post-chemotherapy nadir. Plain abdominal radiography showed some intestinal gas and niveau. Because stool cytotoxin assay for clostridium difficile was positive and colon fiberscopic examination showed a pseudomembrane at the left side of the colon,and a diagnosis of PMC was made. Treatment with intracolonic vancomycin administration by colonoscopy and nasoileus tube was successful. Physicians should take into account the possibility of bowel obstruction due to PMC occurring in patients undergoing chemotherapy and perform emergency colonoscopy examination of suspected cases.

  9. Prognostic Assessment in Patients with Indolent B-Cell Lymphomas

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    Luca Arcaini

    2012-01-01

    Full Text Available Follicular lymphoma (FL is an indolent lymphoma with long median survival. Many studies have been performed to build up prognostic scores potentially useful to identify patients with poorer outcome. In 2004, an international consortium coordinated by the International Follicular Lymphoma Prognostic Factor project was established and a new prognostic study was launched (FLIPI2 using progression-free survival (PFS as main endpoint and integrating all the modern parameters prospectively collected. Low-grade non-Hodgkin lymphomas were once considered as a heterogenous group of lymphomas characterized by an indolent clinical course. Each entity is characterized by unique clinicobiologic features. Some studies have been focused on prognostic factors in single lymphoma subtypes, with the development of specific-entity scores based on retrospective series, for instance splenic marginal zone lymphoma (SMZL. A widely accepted prognostic tool for clinical usage for indolent non-follicular B-cell lymphomas is largely awaited. In this paper we summarized the current evidence regarding prognostic assessment of indolent follicular and non-follicular lymphomas.

  10. Clinicopathological observation of pediatric-type follicular lymphoma occurring in children and adults:a report of three case%发生在儿童及成人的儿童型滤泡性淋巴瘤的临床病理分析

    Institute of Scientific and Technical Information of China (English)

    郑媛媛; 周小鸽

    2014-01-01

    目的探讨发生在儿童及成人的儿童型滤泡性淋巴瘤的临床病例特点。方法对2例典型儿童滤泡性淋巴瘤和1例成人的儿童型滤泡性淋巴瘤进行形态学、免疫组化及克隆性分析,并随访4~18个月。结果3例患者均为男性,发病年龄分别为9岁、14岁及51岁。病变具有儿童滤泡淋巴瘤的典型形态学及临床表现,包括形态上呈现大而扩张的滤泡,显著的满天星现象,组织学呈高级别,具有高的增殖指数,多聚酶链分析显示免疫球蛋白基因单克隆性;病变处于临床Ⅰ期,2例儿童患者经治疗后无复发,1例成人患者未治疗,随访无进展。结论儿童和成人均可发生儿童型FL,具有类似的临床病理特点,预后良好。%Objective To investigate clinicopathological features of pediatric - type follicular lymphoma occurring in children and adults. Methods Pediatric - type follicular lymphoma occurring in two children and one adult were analyzed by light microscopy,immunohistochemistry and polymerase chain reaction(PCR)analysis. These patients were followed for 4 ~ 18 months. Results These three patients included 2 boys and 1 male with the ages of 9,14 and 51. All cases had classic morphologic features of pediatric - type follicular lymphoma,including large,expansile follicles,"starry - sky" pattern,and a high histological grade. Clonal immunoglobulin gene rearrangements were demonstrated by PCR analysis. All patients showed a low - stage disease with excellent outcome. Two pediatric patients received chemotherapy,achieved complete remission, and experienced no relapses. The untreated adult patient that lacked Bcl -2 rearrangement did not progress. Conclusion Pediatric - type FL can occur in both children and adults with similar morphologic,clinical features and a favorable prognosis.

  11. Familial Aggregation of Non-Hodgkin's Lymphoma (NHL. A Case Report

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    Loves Sandra SCM

    2006-08-01

    Full Text Available Abstract A family is reported in which three male siblings of Asian descent developed non-Hodgkin's lymphoma (NHL. Case 1 was diagnosed with indolent follicular lymphoma stage IIIA at age 45. Case 2 presented with large B-cell lymphoma stage IIB at age 56. Chromosomal investigation of the peripheral blood did not show abnormalities. Chemotherapy induced a complete remission. However, after a period of nearly ten years he developed acute myeloid leukaemia. Case 3 developed large B-cell lymphoma stage IVA at age 52. Cytogenetic analysis in peripheral blood was normal. Shared genetic and environmental risk factors remain to be identified in this family. Familial aggregation of NHL is uncommon. In some families, various forms of immunodeficiency have been found. In addition to coincidental clustering of cases, and rare cases explained by known tumour syndromes such as Li-Fraumeni (like syndrome, other familial cases may share as yet unknown genetic and/or environmental risk factors.

  12. Follicular traction urticaria*

    Science.gov (United States)

    Duman, Hatice; Topal, Ilteris Oguz; Kocaturk, Emek

    2016-01-01

    Inducible urticaria is a heterogeneous subgroup of chronic urticarias caused by a wide variety of environmental stimuli, such as exercise, cold, heat, pressure, sunlight, vibration, and water. A new term, follicular traction urticaria, was suggested as an unusual form of inducible urticarias. We report a patient who was diagnosed with follicular traction urticaria.

  13. An uncommon clinical presentation of retroperitoneal non-Hodgkin lymphoma successfully treated with chemotherapy: A case report

    Institute of Scientific and Technical Information of China (English)

    Chiara Fulignati; Pietro Pantaleo; Greta Cipriani; Marianna Turrini; Rosalia Nicastro; Roberto Mazzanti; Bruno Neri

    2005-01-01

    We report the case of apetient affedted by an extra-nodal non-Hodgkin lymphoma presenting as a unique, large retroperitoneal mass with an unusual clinical presentation mimicking gastric peptic or neoplastic disease. The patient was successfully treated with a first generation therapy, CHOP modified regimen (cyclophosphamide 600 mg/m2 intravenously on d 1, epirubicin 55 mg/m2 intravenously on d 1, vincristine 1.2 mg/m2 intravenously on d 1, prednisone 60 mg/m2 on d 1-5), and a complete response was achieved. The (18)F-fluorodeoxyglucose positron emission tomography was used to assess the therapy outcome. A brief review of literature is provided.

  14. Burkitt lymphoma presenting as a mass in the thyroid gland: a clinicopathologic study of 7 cases and review of the literature.

    Science.gov (United States)

    Quesada, Andrés E; Liu, Huifei; Miranda, Roberto N; Golardi, Natalia; Billah, Shahreen; Medeiros, L Jeffrey; Jaso, Jesse Manuel

    2016-10-01

    Burkitt lymphoma presenting in the thyroid gland is rare, and only a few cases have been reported. We retrospectively reviewed 7 patients diagnosed with Burkitt lymphoma of the thyroid gland between 2000 and 2015. There were 4 men and 3 women with a median age of 41 years (range, 19-49 years). All patients presented with a rapidly growing neck mass associated with upper airway compression in 5 (71%) patients. Two patients presented with localized (stage I/II) and 5 patients with disseminated (stage III/IV) disease. All cases showed morphologic and immunophenotypic features of Burkitt lymphoma with MYC rearrangement in all 5 cases tested. One case showed evidence of concurrent Hashimoto thyroiditis. Six of 7 patients were treated primarily with rituximab, hyperfractionated cyclophosphamide, vincristine, doxorubicin, and dexamethasone. One patient was treated primarily with dose-adjusted rituximab, etoposide, prednisolone, vincristine, and cyclophosphamide. At the end of the study period, 5 patients were alive: 4 in complete remission and 1 with persistent disease. Two patients died with persistent disease (median follow-up, 25 months; range, 12-361 months). We conclude that Burkitt lymphoma of the thyroid gland shows clinicopathologic features similar to sporadic Burkitt lymphoma at other anatomic sites, but patients present at an older median age. The clinical course is aggressive with a high frequency of disseminated disease at diagnosis; however, a subset of patients responds well to aggressive chemotherapy.

  15. Reed-Sternberg cells in Hodgkin's lymphoma present features of cellular senescence

    Science.gov (United States)

    Gopas, J; Stern, E; Zurgil, U; Ozer, J; Ben-Ari, A; Shubinsky, G; Braiman, A; Sinay, R; Ezratty, J; Dronov, V; Balachandran, S; Benharroch, D; Livneh, E

    2016-01-01

    Hodgkin's Lymphoma (HL) is one of the most prevailing malignancies in young adults. Reed–Sternberg (RS) cells in HL have distinctive large cell morphology, are characteristic of the disease and their presence is essential for diagnosis. Enlarged cells are one of the hallmarks of senescence, but whether RS cells are senescent has not been previously investigated. Here we show that RS cells have characteristics of senescent cells; RS cells in HL biopsies specifically express the senescence markers and cell cycle inhibitors p21Cip1 and p16INK4a and are negative for the proliferation marker Ki-67, suggesting that these cells have ceased to proliferate. Moreover, the RS-like cells in HL lines, stained specifically for senescence-associated β-galactosidase (SA-β-gal). Oxidative stress promoted senescence in these cells as demonstrated by their staining for p21Cip1, p16INK4a, p53 and γH2AX. Senescent cells produce copious amounts of inflammatory cytokines termed ‘senescence-associated secretory phenotype' (SASP), primarily regulated by Nuclear Factor κB (NF-κB). Indeed, we show that NF-κB activity and NF-κB-dependent cytokines production (e.g., IL-6, TNF-α, GM-CSF) were elevated in RS-like cells. Furthermore, NF-κB inhibitors, JSH-23 and curcumin reduced IL-6 secretion from RS-like cells. Thus, defining RS cells as senescent offers new insights on the origin of the proinflammatory microenvironment in HL. PMID:27831553

  16. Reed-Sternberg cells in Hodgkin's lymphoma present features of cellular senescence.

    Science.gov (United States)

    Gopas, J; Stern, E; Zurgil, U; Ozer, J; Ben-Ari, A; Shubinsky, G; Braiman, A; Sinay, R; Ezratty, J; Dronov, V; Balachandran, S; Benharroch, D; Livneh, E

    2016-11-10

    Hodgkin's Lymphoma (HL) is one of the most prevailing malignancies in young adults. Reed-Sternberg (RS) cells in HL have distinctive large cell morphology, are characteristic of the disease and their presence is essential for diagnosis. Enlarged cells are one of the hallmarks of senescence, but whether RS cells are senescent has not been previously investigated. Here we show that RS cells have characteristics of senescent cells; RS cells in HL biopsies specifically express the senescence markers and cell cycle inhibitors p21(Cip1) and p16(INK4a) and are negative for the proliferation marker Ki-67, suggesting that these cells have ceased to proliferate. Moreover, the RS-like cells in HL lines, stained specifically for senescence-associated β-galactosidase (SA-β-gal). Oxidative stress promoted senescence in these cells as demonstrated by their staining for p21(Cip1), p16(INK4a), p53 and γH2AX. Senescent cells produce copious amounts of inflammatory cytokines termed 'senescence-associated secretory phenotype' (SASP), primarily regulated by Nuclear Factor κB (NF-κB). Indeed, we show that NF-κB activity and NF-κB-dependent cytokines production (e.g., IL-6, TNF-α, GM-CSF) were elevated in RS-like cells. Furthermore, NF-κB inhibitors, JSH-23 and curcumin reduced IL-6 secretion from RS-like cells. Thus, defining RS cells as senescent offers new insights on the origin of the proinflammatory microenvironment in HL.

  17. A Case Report of Primary Nasal Natural Killer (NK)/T-Cell Lymphoma in an African American Patient Presenting with Hemophagocytic Syndrome

    Science.gov (United States)

    Tan, Bowei; Abdelmalek, Cherif; O’Donnell, James E.; Toltaku, Thomas; Chaudhry, Rashid; Wang, Jen C.; Gotlieb, Vladimir

    2017-01-01

    Patient: Male, 55 Final Diagnosis: Primary NK-T cell lymphoma • nasal type Symptoms: Fever • nasal bleeding • nasal mass • weight loss Medication: — Clinical Procedure: Chemotherapy×2 cycles • radiation therap Specialty: Oncology Objective: Rare disease Background: Extranodal natural killer/T-cell lymphoma, nasal type (ENKTCL) is generally an aggressive and rare non-Hodgkin lymphoma. It is most common in East Asians, Native Americans, and South Americans, but is rarely reported in blacks. Case Report: A 55-year-old African American male born in Grenada presented with a left nostril mass with facial swelling and biopsy subsequently confirmed a diagnosis of extranodal NK/T-cell lymphoma, nasal type (ENKTCL). Immunochemistry was positive for CD2, cytoplasmic CD3, CD7, CD 43, CD 56, granzyme B, and TIA-1. In situ hybridization was positive for Epstein-Barr virus encoded ribonucleic acid (EBERs). Bone marrow aspiration did not show lymphoma involvement. The patient had progressive neutropenia upon presentation, with further investigations showing hepatomegaly, hyperferritinemia, and hemophagocytosis in the bone marrow. We reached a diagnosis of hemophagocytic syndrome. He was treated with a high-dose combination chemotherapy and radiation therapy; the neutropenia improved significantly with steroids as treatment for immune activation in the setting of hemophagocytic syndrome. Conclusions: To the best of our knowledge, this is the only second report of extranodal NK/T-cell lymphoma, nasal type in a black patient, and it raises the awareness of early recognition of rare manifestations of NK/T-cell lymphoma such as hemophagocytic syndrome. PMID:28193996

  18. Fever of unknown origin (FUO) in an elderly adult due to Epstein-Barr virus (EBV) presenting as "typhoidal mononucleosis," mimicking a lymphoma.

    Science.gov (United States)

    Cunha, Burke A; Petelin, Andrew; George, Sonia

    2013-01-01

    We describe fever of unknown origin (FUO) in a 57-year-old woman with hepatosplenomegaly. The diagnostic workup was directed at diagnosing a lymphoma. Her history of travel and exposures to food and water did not make typhoid fever a likely diagnostic possibility. Because she presented with prolonged fevers, fatigue, anorexia, weight loss, and night sweats with hepatosplenomegaly, lymphoma was likely. Initially, Epstein-Barr virus (EBV) was not considered because of her age, the absence of pharyngitis and cervical adenopathy, and the higher likelihood of another diagnosis, ie, lymphoma. Eventually, her FUO was diagnosed as EBV presenting as "typhoidal mononucleosis." Typhoidal mononucleosis is an extremely rare presentation of EBV as a cause of FUO in an adult. All of her symptoms as well as her clinical and laboratory findings resolved spontaneously. Copyright © 2013 Elsevier Inc. All rights reserved.

  19. Clinical Characteristics of Malignant Lymphoma with Retroperitoneal Mass as the Primary Presentation%腹膜后肿块为首发症状的恶性淋巴瘤临床特点分析

    Institute of Scientific and Technical Information of China (English)

    金哈斯; 宫丽平; 倪美兰; 孟文霞; 林美雄; 母立新

    2012-01-01

    目的:探讨腹膜后肿块为首发症状的恶性淋巴瘤的临床特点、治疗及预后.方法:回顾性收集自2002年1月至2010年12月间收治的20例腹膜后肿块为首发症状的恶性淋巴瘤患者的临床资料,分析其病理活检方式、病理类型、免疫组化、分期、治疗效果、生存率等.研究手术、年龄、性别、分期、巨大包块、B症状、病理类型对近期疗效的影响.结果:20例患者中11例(55%)为弥漫大B细胞性淋巴瘤,4例(20%)为滤泡性淋巴瘤,2例(10%)为小淋巴细胞性淋巴瘤/慢性淋巴细胞白血病,1例(5%)为淋巴母细胞性淋巴瘤,1例(5%)为间变细胞淋巴瘤,1例(5%)霍奇金淋巴瘤.全组患者化疗后完全缓解7例(35%),部分缓解8例(40%);5例就诊后3个月之内早期死亡,9例起病后4~36个月之间疾病进展或复发死亡.全组患者中5年生存率仅20%.手术、病理类型、年龄、大包块、浸润周围器官广泛、静脉血栓形成、肝功能异常、B症状对近期疗效有明显影响.结论:腹膜后肿块为首发症状的恶性淋巴瘤缓解率和长期生存率较低,预后差.病理类型、手术、大包块、浸润周围器官、肝功能异常、静脉血栓形成、B症状对缓解率和长期生存率有明显影响.%Objective: To investigate the clinical characteristics,treatment outcomes and prognostic factors of patients with malignant lymphoma with primary retroperitoneal mas as the primary presentation. Methods: A total 20 cases of malignant lymphoma with primary retroperitoneal presentation lymphoma seen in our hospital from January 2002 to Decemter 2010 Biopsy method , tissue pathology, immunochemistry,treatmemt outcome and long time survival were analyzed retrospectively. The influences of age, sex, pathologic type, clinical stage, huge mass and B symptom were analyzed. Results: Among the 20 cases, 11 ( 55 % ) were diffuse large B cell lymphoma, 4 ( 20 % )were follicular lymphoma, 2 ( 10 % ) were B

  20. Diffuse large B-cell non Hodgkin's lymphoma in a 65-year-old woman presenting with hypopituitarism and recovering after chemotherapy: a case report

    Directory of Open Access Journals (Sweden)

    Hyer Steve L

    2011-10-01

    Full Text Available Abstract Introduction Diffuse large B-cell non Hodgkin's lymphoma may involve the pituitary either as a primary central nervous system lymphoma or, more frequently, as metastasis from systemic lymphoma leading to hypopituitarism. A partial recovery of pituitary function after treatment with chemotherapy has previously been described but complete recovery with cessation of all hormone supplements is excessively rare. We report a patient presenting with anterior hypopituitarism with subsequent complete and sustained recovery of pituitary function after successful treatment of the lymphoma. Case presentation A 65-year-old Caucasian woman with lethargy, loss of appetite and peripheral edema was found to have anterior hypopituitarism. Magnetic resonance imaging showed no mass lesions in the pituitary although a positron emission tomography scan showed abnormal pituitary activity. An abdominal computed tomography scan revealed multiple intra-abdominal lymph nodes, which on histology proved diagnostic of diffuse large B-cell non Hodgkin's lymphoma. She received six cycles of R-CHOP chemotherapy, after which she achieved a complete metabolic response at all known previous sites of the disease, confirmed by positron emission tomography scanning. Concomitant with the tumor response, there was full recovery of adrenal, thyroid and gonadal axes which has persisted at 10 months follow-up. Conclusion Although rare, it is important to recognize lymphomatous infiltration of the pituitary as a potentially reversible cause of hypopituitarism.

  1. Diffuse large B-cell lymphoma: clinical implications of extranodal versus nodal presentation--a population-based study of 1575 cases

    DEFF Research Database (Denmark)

    Møller, Michael B; Pedersen, Niels T; Christensen, Bjarne E

    2004-01-01

    Differences in genetic origin between nodal and extranodal diffuse large B-cell lymphomas (DLBCL) exist. Using population-based data from the registry of the Danish Lymphoma Group, the present study is the first to analyse clinical implications of nodal versus extranodal presentation of DLBCL....... Of 4786 newly diagnosed non-Hodgkin's lymphoma patients in a 16-year period, 1575 (33%) had DLBCL. The annual incidence rate was 2.9 per 100 000; 40% were extranodal. The clinical profile of patients with extranodal DLBCL was different from the nodal DLBCL patients. Extranodal DLBCL was associated...... with older age and poorer performance score, but also lower tumour burden. In extranodal DLBCL, 51% of the cases were stage I and 36% were stage IV, whereas the patients were relatively equally distributed between the four stages in nodal DLBCL. For stage I patients, extranodal DLBCL was independently...

  2. Non-Hodgkin′s lymphoma of the hard palate

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    Jayakrishnan R

    2008-01-01

    Full Text Available Background: Non-Hodgkin′s lymphoma usually involves lymph nodes but can also involve extranodal sites. Oral lymphomas are relatively rare and often difficult to diagnose in a clinical setting. We present a case of a patient of non-Hodgkin′s lymphoma of the hard palate who had undergone external beam radiation therapy and was found to be well 1 year following treatment. Case presentation: A 43-year-old male attended the Community Oncology Division of Regional Cancer Centre with complaints of painless swelling on the right side of face of three months′ duration. A computed tomography (CT scan confirmed irregular destruction of the hard palate towards the right side of the midline with adjacent soft tissue component. Histopathology report confirmed the diagnosis of follicular lymphoma. He was given radical external beam radiation therapy (40 Gy in 20 fractions over 4 weeks. Clinical examination at the end of radiation revealed complete disappearance of the palatal swelling. Conclusion: Oral lymphomas are relatively rare and often difficult to diagnose as the clinical features mimic other pathological entities such as periodontal disease, osteomyelitis, and other malignancies. A careful clinical evaluation supported by radiologic and histopathologic investigations will help in identifying the disease at an early stage, which will result in better prognosis.

  3. Follicular occlusion tetrad

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    Vani Vasanth

    2014-01-01

    Full Text Available Follicular occlusion tetrad is a symptom complex consisting of four conditions having a similar pathophysiology. It includes Hidradenitis suppurativa, acne conglobata, dissecting cellulitis of the scalp and pilonidal sinus. The exact pathogenesis of this group of disease is unknown but evidence suggests that they share the same pathological process initiated by follicular occlusion in apocrine gland bearing areas. Though each of these conditions is commonly encountered singly, follicular occlusion tetrad as a symptom complex has been rarely reported in the literature.

  4. Ongoing trials in low-grade lymphoma

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    Alexander Burchardt

    2011-10-01

    Full Text Available There are many therapies available for the management of low-grade lymphoma. With follicular lymphoma, for example, combination of chemotherapy and rituximab (immuno-chemo - therapy and consecutive maintenance therapy for 2 years is the current standard of care. To date, the most widely used regimen seems to be rituximab combined with cyclo phosphamide, doxorubicin, vincristine, and prednisone (RCHOP. Substitution of liposomal doxorubicin in place of conventional doxorubicin may improve outcomes in this indication, although evidence for its use in low-grade lymphoma is not as relevant as in aggressive lymphoma. Bendamustine, in combination with rituximab, has shown very good efficacy and tolerability in several lymphoma types, particularly follicular lymphoma and other low-grade lymphomas. Other combinations, such as those including bortezomib and lenalidomide, are under investigation in low-grade lymphoma, and the duration of rituximab maintenance therapy following bendamustine−rituximab-containing induction is being researched by the German Study Group for Indolent Lymphoma (StiL.

  5. Lymphoma cytogenetics.

    Science.gov (United States)

    Dave, Bhavana J; Nelson, Marilu; Sanger, Warren G

    2011-12-01

    Lymphomas are a heterogeneous group of neoplasms with distinct morphologic, immunologic, and cytogenetic characteristics. Overlapping morphologic and immunophenotypic features often makes accurate diagnosis difficult. Cytogenetics helps simplify the diagnostic complexities presented in transforming and progressive lymphoid malignancies. Genetic studies using technical advances such as fluorescence in situ hybridization and the newer approaches of array comparative genomic hybridization and gene expression profiling play a critical and often defining role in the diagnosis, progression, prognosis, and therapeutic stratification. This article reviews characteristic cytogenetic abnormalities in specific subtypes of lymphomas at diagnosis, disease progression, and prognosis.

  6. Non-Hodgkin's lymphoma presenting as a single liver mass; Linfoma nao-Hodgkin apresentando-se como massa hepatica unica

    Energy Technology Data Exchange (ETDEWEB)

    Peixoto, Mila Correia Gois; Peixoto Filho, Anibal Araujo Alves; D' Ippolito, Giuseppe [Hospital Sao Luiz, Sao Paulo, SP (Brazil). Setor de US/TC/RM]. E-mail: scoposl@uol.com.br; Ribeiro, Alessandra Caivano Rodrigues [Hospital Sao Luiz, Sao Paulo, SP (Brazil). Setor de Diagnostico por Imagem

    2009-01-15

    Objective: to describe the main imaging findings of non-Hodgkin's lymphoma presenting as a single liver mass. Materials and methods: a retrospective study was developed with analysis of cases where a single liver mass was observed at ultrasonography, computed tomography and magnetic resonance imaging, and histologically diagnosed as non-Hodgkin's lymphoma. The studies were reviewed by two observers in consensus. Results: three male patients in the fifth decade of life, with non-specific clinical manifestations and single liver mass diagnosed as non-Hodgkin's lymphoma were identified. A hepatic lesion with target sign was observed at ultrasonography in all of the cases. At computed tomography, all the patients presented a heterogeneous, hypodense mass with a ring enhancement. At magnetic resonance imaging, the lesions were heterogeneous and hypointense on T1-weighted and hyperintense on T2-weighted images. Additionally, a ring enhancement was observed in all of the cases after contrast injection. At the moment of the diagnosis, none of the patients presented lymphadenomegaly or involvement of other solid viscera. Conclusion: the diagnosis of hepatic lymphoma should be considered in the presence of a ring-enhanced single liver mass. (author)

  7. Angiogenesis in non-Hodgkin's lymphoma: clinico-pathological correlations and prognostic significance in specific subtypes

    DEFF Research Database (Denmark)

    Jørgensen, J M; Sørensen, Flemming Brandt; Bendix, K;

    2007-01-01

    The aim of the study was to evaluate angiogenesis in different subtypes of non-Hodgkin's lymphoma (NHL) and to correlate angiogenic scores to clinical endpoints. Pre-therapeutic lymph node biopsies from 308 patients with NHL [107 follicular B-cell lymphoma (FL), 94 diffuse large B-cell lymphoma (...

  8. Hodgkin's lymphoma presenting as dominant gastric lesion in immunocompetent patients: report of 5 cases with EBV analysis.

    Science.gov (United States)

    Gualco, Gabriela; Ortega, Virginia; Chioato, Lucimara; Musto, Maria L; Bacchi, Livia M; Weiss, Lawrence M; Bacchi, Carlos E

    2011-04-01

    Primary Hodgkin's lymphoma (HL) of the stomach is an extremely rare entity. Most cases of gastric involvement by HL are observed in the setting of disseminated disease. The nonspecific nature of the symptoms and endoscopic findings, which include a large malignant-looking ulcer and mass or wall thickening, together with the considerable histological overlap between HLs and some non-HLs or undifferentiated carcinoma, make the surgical resection diagnosis extremely difficult. An accurate diagnosis is important as treatment and outcome differ significantly for these neoplasms. In small endoscopic gastric biopsies and even in postoperative specimens, the precise histological diagnosis of HL is particularly challenging. Here, the authors report 5 cases of 2 women and 3 men aged 22 to 68, with gastric involvement by classic HLs-3 primary gastric HLs and 2 as part of widespread disease. All 5 patients presented with digestive symptoms. At endoscopy, the lesions presented as ulcerated and elevated lesions, with or without mucosal thickening. Four patients were misdiagnosed in the preoperative biopsy or in the gastrectomy specimen. Association with Epstein-Barr virus (EBV) was detected in 4 cases, with a predominance of subtype A EBV. These cases illustrate the significant difficulties, both clinical and pathological, in achieving the diagnosis of HL involving the stomach in immunocompetent patients.

  9. COAGULATION PROFILE IN PATIENTS PRESENTING WITH MALIGNANCIES WITH SPECIAL REFERENCES TO HEAD AND NECK EPITHELIAL CANCERS, LEUKAEMIAS AND LYMPHOMAS

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    Kaberee Bhuyan

    2016-04-01

    Full Text Available BACKGROUND Cancer can cause activation of coagulation in many ways and there is definite evidence of abnormalities in haemostatic mechanism which is seen by the presence of one or more circulating markers of haemostatic activation & this is found to be potentiated by the release of tissue factors or procoagulants from normal tissue destructions during tumour development. OBJECTIVES • To evaluate the range of different types of haemostatic abnormalities in haematological and epithelial malignancies, especially the head and neck epithelial malignancies. • To look for the differences in the grades of these abnormalities in metastatic & non-metastatic malignancies. • To understand the prognostic value of routine tests of coagulation while predicting the outcome of the patient. • MATERIALS AND METHODS The study was conducted in the Department of Pathology, Gauhati Medical College & Hospital, Guwahati from July 2004 to June 2005. 70 cases comprising of head and neck epithelial malignancies, leukaemias and lymphomas without clinical presentation of haemorrhage or thrombosis were selected and coagulation profiles were seen. RESULTS AND OBSERVATION Out of 70 cases of both sexes & different age groups prior to therapeutic intervention, metastatic cases were 22, non-metastatic cases were 29, and 19 cases belonged to leukaemias and lymphomas. The commonest age group affected was 51–60 yrs. and male: female was 3.7: 1. The most frequent abnormality was 41 cases (58.57% of FDP positivity in the serum followed by 36 cases (51.43% of hyperfibrinogenaemia; 32 cases (45.71% shortened bleeding time, etc. DISCUSSION Activated coagulation in cancer leads to increased fibrin deposition stimulated by the destroyed tissues; increased FDPs being a strong marker of coagulation and fibrinolytic activation; increased platelet aggregation by the micro vesicles shed by tumour cells; prolonged PT & APTT being well known markers for disseminated intravascular

  10. Delayed Recognition of a Rare Mediastinal Lymphoma Presenting as Postpartum Circulatory Collapse

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    Jasmina Kevric

    2014-01-01

    Full Text Available We report a case of a 29-year-old primigravida at 36 weeks of gestation following an emergency caesarean section, complicated by respiratory distress and multiorgan failure secondary to superior vena cava (SVC obstruction, requiring intubation and prolonged ventilatory support. The presented case highlights the consequences of delayed recognition of SVC obstruction due to a reluctance to undertake appropriate radiological imaging during pregnancy.

  11. Renal small B-cell lymphoma with plasmacytic differentiation presenting with monoclonal gammopathy and disseminated intravascular coagulation syndrome

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    Paula de Oliveira Pádua Prestes

    2013-10-01

    Full Text Available Primary renal lymphomas are very rare. However, the kidney may be a site of metastasis, usually from a disseminated aggressive lymphoma. A 58-year-old woman was brought to the medical facility due to acute mental confusion, severe hypotension, septic shock, and signs of disseminated intravascular coagulation. Laboratory tests showed severe leukopenia, renal failure, altered liver function, and elevated serum lactate dehydrogenase levels. Protein electrophoresis revealed hypergammaglobulinemia with a monoclonal peak of IgG lambda. The clinical outcome was fulminant and the patient died less than 24 hours after admission. Autopsy revealed an indolent B-cell lymphoma with extensive plasmacytic differentiation infiltrating the right renal sinus and involving the submandibular and sublingual glands, cervical and peri-aortic lymph nodes, multiple microscopic foci in pituitary and adrenal glands, lung, breast, liver, thyroid, and bone marrow. Numerous IgG4-positive plasma cells were detected by immunohistochemistry although other histological features of IgG4-related disease were missing. There was also extensive hemorrhagic necrosis of the adrenal glands and purulent cystitis, which was probably responsible for the septic shock. The authors concluded that the kidney was most likely the primary site of the indolent lymphoma, as that was the site with the largest tumor mass. Infiltration of other organs was considered as dissemination of the disease. The differential diagnosis with mucosa-associated lymphoid tissue and lymphoplasmacytic lymphoma is discussed.

  12. Acute kidney injury in the setting of AIDS, bland urine sediment, minimal proteinuria and normal-sized kidneys: a presentation of renal lymphoma.

    Science.gov (United States)

    Sandhu, Gagangeet; Ranade, Aditi; Mankal, Pavan; Herlitz, Leal C; Jones, James; Cortell, Stanley

    2011-02-01

    Acute kidney injury in HIV patients is primarily related to HIV-mediated viral or immunological disease or to treatment-related toxicity (tenofovir). Neoplasms are a rare cause of non-obstructive acute kidney injury, primarily because when they occur, they manifest as discrete masses and not as diffuse infiltration of the renal parenchyma. Diffusely infiltrating tumors include carcinoma of the renal pelvis invading the renal parenchyma, renal lymphoma, squamous cell carcinoma (from lung) metastasizing to the kidney and infiltrating sarcomatous type of renal cell carcinoma. To be classified as a true case of renal lymphoma, the tumor should have escaped detection on routine imaging preceding biopsy, and lymphoma-associated renal failure/nephrotic proteinuria should have given rise to the indication for kidney biopsy. We present here a case of an acute kidney injury due to renal lymphoma in a patient with acquired immune deficiency syndrome that manifested clinically as bland urine sediment, minimal proteinuria and normal-sized kidneys. Chemotherapy resulted in complete reversal of acute kidney injury.

  13. Polymyalgia Rheumatica Revealing a Lymphoma: A Two-Case Report

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    Frank Verhoeven

    2016-01-01

    Full Text Available Introduction. Polymyalgia rheumatica (PMR is one of the most common inflammatory rheumatism types in elderly population. The link between cancer and PMR is a matter of debate. Methods. We report two cases of PMR leading to the diagnosis of lymphoma and the growing interest of PET-TDM in this indication. Results. A 84-year-old man known for idiopathic neutropenia presented an inflammatory arthromyalgia of the limb girdle since one month. Blood exams highlighted the presence of a monoclonal B cell clone. Bone marrow concluded to a B cell lymphoma of the marginal zone. He was successfully treated with 0.3 mg/kg/d of prednisone, and response was sustained after 6 months. A 73-year-old man known for prostatic neoplasia in remission for 5 years presented arthromyalgia of the limb girdle since one month. PET-CT revealed bursitis of the hips and the shoulders, no prostatic cancer recurrence, and a metabolically active iliac lymphadenopathy whose pathologic exam concluded to a low grade follicular lymphoma. He was successfully treated with 0.3 mg/kg/d of prednisone. Conclusion. These observations may imply that lymphoma is sometimes already present when PMR is diagnosed and PET-CT is a useful tool in the initial assessment of PMR to avoid missing neoplasia.

  14. Acute spontaneous tumor lysis in anaplastic large T-cell lymphoma presenting with hyperuricemic acute renal failure.

    Science.gov (United States)

    Hsu, Hsiang-Hao; Huang, Chiu-Ching

    2004-01-01

    Acute spontaneous tumor lysis (ASTL) syndrome, an extremely rare disease, requires prompt recognition and aggressive management because it is fulminant at its outset, associated with severe metabolic derangement, and potentially reversible. We describe an unusual case in which spontaneous tumor lysis occurred in anaplastic large T-cell lymphoma associated with acute uric acid nephropathy, persistent oliguria, and shock. This case contrasts markedly with previously reported cases of ASTL syndrome, which developed mainly in the pathologic type of Burkitt lymphoma. To our knowledge, this is the first reported occurrence of ASTL syndrome associated with anaplastic large T-cell type lymphoma. This report also chronicles our successful experience with continuous renal replacement therapy in the presence of compromised hemodynamic status.

  15. Complete Heart Block and Persistent Lactic Acidosis as an Initial Presentation of Non-Hodgkin Lymphoma in a Critically Ill Newly Diagnosed AIDS Patient

    Directory of Open Access Journals (Sweden)

    Mohsin Ijaz

    2014-01-01

    Full Text Available A 66-year-old male with newly diagnosed untreated acquired immunodeficiency syndrome (AIDS presented with chronic nonspecific complaints of weakness, fatigue, myalgia, and weight loss. His initial EKG showed complete heart block necessitating temporary pacemaker placement. He had no previous history of cardiac disease. He was also found to have a persistent lactic acidosis and imaging studies showed abdominal lymphadenopathy. The patient underwent biopsy of these lymph nodes and was found to have diffuse large B-cell lymphoma. The hospital course was complicated by respiratory failure requiring mechanical ventilator support and cardiac arrest. Patient remained critically ill; he was not a candidate for chemotherapy and, after a month of hospitalization, he died. Lactic acidosis and heart block as an initial presentation of non-Hodgkin lymphoma in an AIDS patient are an unusual and unique presentation.

  16. Bifocal Presentation of Primary Testicular Extranasal NK/T-Cell Lymphoma: A Case Report and Review of the Literature

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    Ali Naboush

    2013-01-01

    Full Text Available Introduction. Testicular lymphoma is an aggressive disease with a very poor prognosis. Nasal-type natural killer/T-cell lymphoma (NKTCL-N in particular is very uncommon and has a rapidly progressive, fatal course. Case Report. We report a case of primary NKTCL-N of the testis in a 38-years-old Middle Eastern man. The patient had a history of primary right testicular tumor diagnosed at an outside institution as a seminoma and treated with orchiectomy followed by chemo/radiation. On admission, the patient had an enormous nasal granuloma with blood workup showing pancytopenia and elevated liver function tests due to active hepatitis B infection. CT scan of the sinuses showed a very large soft tissue mass, and PET scan showed splenomegaly with multiple lymph node masses in the pelvis and the chest areas. Bone marrow and nasal tumor biopsies as well as review of the slides from the initial orchiectomy were all in favor of NKTCL-N lymphoma. The patient was treated with CHOD based combination chemotherapy and responded dramatically to the first two cycles but passed away from fulminant hepatitis B infection. Conclusion. Despite all known treatments of NKTCL-N lymphoma of the testes, this disease has a very poor prognosis and invariably follows an aggressive clinical course.

  17. Prevalence of Borrelia burgdorferi infection in a series of 98 primary cutaneous lymphomas.

    Science.gov (United States)

    Ponzoni, Maurilio; Ferreri, Andrés J M; Mappa, Silvia; Pasini, Elisa; Govi, Silvia; Facchetti, Fabio; Fanoni, Daniele; Tucci, Alessandra; Vino, Arianna; Doglioni, Claudio; Berti, Emilio; Dolcetti, Riccardo

    2011-01-01

    Borrelia burgdorferi has been variably associated with different forms of primary cutaneous lymphoma. Differences in prevalence rates among reported studies could be a result of geographic variability or heterogeneity in the molecular approaches that have been employed. In the present study, we investigated the prevalence of Borrelia burgdorferi sensu lato DNA in diagnostic tissue samples from fresh cutaneous biopsies of 98 primary cutaneous lymphomas and 19 normal skin controls. Three different polymerase chain reaction (PCR) protocols targeting the hbb, flagellin, and Osp-A genes were used. Direct sequencing of both sense and antisense strands of purified PCR products confirmed the specificity of the amplified fragments. Sequence specificity was assessed using the Basic Local Alignment Search Tool, and MultAlin software was used to investigate the heterogeneity of target gene sequences across the different samples. Borrelia DNA was not detected in 19 controls, 23 cases of follicular lymphoma, 31 cases of extranodal marginal zone lymphoma, or 30 cases of mycosis fungoides. A single case of 14 diffuse large B-cell lymphoma cases was positive for B. burgdorferi. This study does not support a pathogenic role of B. burgdorferi in primary cutaneous B- and T-cell lymphomas from areas nonendemic for this microorganism and the consequent rationale for the adoption of antibiotic therapy in these patients.

  18. Follicular occlusion triad

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    Mittal R

    1993-01-01

    Full Text Available One 40-year female was diagnosed as a case of follicular occlusion triad (FOT as she had hidradenitis suppurativa, acne conglobata and perifolliculitis capitis abscedens et suffodiens since 7 years. Partial improvement occurred within 20 days of treatment with co-trimoxazole and haematinics but relapse occurred after 5 days of stopping antibiotics.

  19. Post cardiac transplantation lymphoproliferative disorder presenting as t(8;14) Burkitt leukaemia/lymphoma treated with low intensity chemotherapy and rituximab.

    Science.gov (United States)

    Windebank, Kevin; Walwyn, Tom; Kirk, Richard; Parry, Gareth; Hasan, Asif; Bown, Nick; Wilkins, Bridget

    2009-09-01

    Post-transplant lymphoproliferative disorder (PTLD) occasionally presents as Burkitt lymphoma/L3 leukaemia (BLL). We reviewed records of cases of PTLD post-cardiac transplantation (1990-2007) occurring in our unit. There were 15 episodes in 13 patients including four cases of EBV-driven Burkitt-type disease with t(8;14) translocations presenting with advanced stage disease. The first case was treated with a variety of low dose chemotherapy combinations. Despite problems during therapy he obtained complete remission, but died from complications of pre-existing cardiac allograft vasculopathy 7 months later. The subsequent three cases were treated with a UKCCSG low stage lymphoma protocol, NHL 9001 and Rituximab. They remain in complete remission. In the context of PTLD the prognostic significance of advanced stage EBV-driven BLL with the t(8;14) translocation may be different to that in immunocompetent children. (c) 2009 Wiley-Liss, Inc.

  20. Prospective, multicenter randomized GITMO/IIL trial comparing intensive (R-HDS) versus conventional (CHOP-R) chemoimmunotherapy in high-risk follicular lymphoma at diagnosis: the superior disease control of R-HDS does not translate into an overall survival advantage.

    Science.gov (United States)

    Ladetto, Marco; De Marco, Federica; Benedetti, Fabio; Vitolo, Umberto; Patti, Caterina; Rambaldi, Alessandro; Pulsoni, Alessandro; Musso, Maurizio; Liberati, Anna M; Olivieri, Attilio; Gallamini, Andrea; Pogliani, Enrico; Rota Scalabrini, Delia; Callea, Vincenzo; Di Raimondo, Francesco; Pavone, Vincenzo; Tucci, Alessandra; Cortelazzo, Sergio; Levis, Alessandro; Boccadoro, Mario; Majolino, Ignazio; Pileri, Alessandro; Gianni, Alessandro M; Passera, Roberto; Corradini, Paolo; Tarella, Corrado

    2008-04-15

    In this randomized multicenter study of 136 patients, 6 courses of CHOP (cyclo-phosphamide/doxorubicin/vincristine/prednisone) followed by rituximab (CHOP-R) were compared with rituximab-supplemented high-dose sequential chemotherapy with autografting (R-HDS) to assess the value of intensified chemo-therapy as a first-line treatment for high-risk follicular lymphoma (FL) after the introduction of monoclonal antibodies. The analysis was intention to treat with event-free survival (EFS) as the primary endpoint. Complete remission (CR) was 62% with CHOP-R and 85% with R-HDS (P HDS patients (P HDS in 71% of cases. Salvage R-HDS had an 85% CR rate and a 68% 3-year EFS (MFU, 30 months). We conclude that (1) achieving MR is critical for effective disease control, regardless of which treatment is used; (2) R-HDS ensures superior disease control and molecular outcome than CHOP-R, but no OS improvement; and (3) CHOP-R failures have a good outcome after salvage R-HDS, suggesting that relapsed/refractory FL could be the most appropriate setting for R-HDS-like treatments. This trial was registered at www.clinicaltrials.gov as no. NCT00435955.

  1. Expression of p63 in anaplastic large cell lymphoma but not in classical Hodgkin's lymphoma.

    Science.gov (United States)

    Gualco, Gabriela; Weiss, Lawrence M; Bacchi, Carlos E

    2008-10-01

    Immunohistochemical determination of p63 protein is frequently used in the pathologic diagnosis of nonhematological solid tumors. In malignant hematological disease, p63 expression has been reported in 22% of follicular lymphoma, about 35% of diffuse large B-cell lymphoma, 23% of chronic lymphocytic leukemia, and in some cases of blast crisis of chronic myelogenous leukemia. Anaplastic large cell lymphoma is a rare disease that accounts for less than 5% of all cases of non-Hodgkin's lymphoma. There is little information concerning p63 expression in this specific type of lymphoma. In some cases, the morphological and phenotypic features between anaplastic large cell lymphoma and classical Hodgkin's lymphoma are similar, making this differential diagnosis challenging. We studied p63 expression using a tissue microarray approach in 154 cases of anaplastic large cell lymphoma, including 38% anaplastic large cell kinase positive and 62% anaplastic large cell kinase negative, and 58 Hodgkin's lymphoma cases. Sixty-eight cases of anaplastic large cell lymphoma (44%) showed p63 nuclear positivity (41% of anaplastic large cell kinase positive and 47% of anaplastic large cell kinase negative). Of 130 cases of systemic-anaplastic large cell lymphoma, 42% showed p63 positivity. The neoplastic cells expressed p63 in 38% of the cases of CD45-negative/anaplastic large cell kinase-negative null cell-type anaplastic large cell lymphoma, a subgroup that offers the most difficulties in the differential diagnosis with classical Hodgkin's lymphoma. In contrast, none of the cases of classical Hodgkin's lymphoma demonstrated any p63 expression. These results demonstrate that p63 protein expression is frequently expressed in a subset of anaplastic large cell lymphoma cases and may be used as a potential tool in the differential diagnosis between anaplastic large cell lymphoma and classical Hodgkin's lymphoma.

  2. Intravascular Large B Cell Lymphoma Presenting as Fever of Unknown Origin and Diagnosed by Random Skin Biopsies: A Case Report and Literature Review.

    Science.gov (United States)

    di Fonzo, Horacio; Contardo, Damian; Carrozza, Diego; Finocchietto, Paola; Rojano Crisson, Adriana; Cabral, Cecilia; de Los Angeles Juarez, Maria

    2017-05-02

    BACKGROUND Intravascular lymphoma (IVL) is a rare lymphoproliferative disorder characterized by the proliferation of large B lymphoma cells within the lumen of small-caliber blood vessels. Clinical features are nonspecific, presenting as a systemic disease with fever and may be life-threatening. Antemortem diagnosis is difficult but may be made with biopsies of affected tissues or with random skin biopsies. CASE REPORT We report the case of a 66-year-old white woman presenting with fever of unknown origin (FUO) who developed neurologic, pulmonary, and hematologic manifestations. The diagnosis of intravascular large B cell lymphoma (IVLBCL) was made by random skin biopsies. She received treatment with steroids, rituximab, cyclophosphamide, vincristine, and doxorubicin (R-CHOP). Her disease evolution was unfavorable and she died after her first cycle of chemotherapy. CONCLUSIONS Our case illustrates that IVL can present as FUO and should be considered in the differential diagnosis of this syndrome, especially in patients with neurologic compromise and persistently elevated serum lactate dehydrogenase. In this case, the diagnosis was made with cutaneous biopsies of visibly unaffected skin. As in our patient, the course of IVL is usually fatal within a few months.

  3. Malignant Lymphoma in an Atomic-bomb Survivor

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    Cheng-Chia Lee

    2009-07-01

    Full Text Available Atomic bomb survivors outside of Japan are few and often hard to follow-up. Spinal malignant lymphoma among these survivors is rare in established studies from Japan or the United States. Here, we report an 81-year-old woman, who experienced the atomic bomb explosion in Nagasaki when she was 19 years old, who presented with papillary thyroid carcinoma when she was 70 years old. Both follicular lymphoma over the right elbow region and vertebral malignant lymphoma were found when she turned 81 years old. Bone scan did not show any increased uptake of isotope. However, thoracolumbar spine magnetic resonance imaging showed multiple infiltrative soft tissue masses involving vertebral bodies at the T10–11 level. Computed tomography-guided biopsy further showed lymphocyte infiltration. Fortunately, the neurological deficit was improved after chemotherapy. The diagnosis of malignant lymphoma in atomic bomb survivors should be more careful and aggressive, even when their bone scan results show negative findings. In addition, the authors suggest that atomic bomb survivors should be followed-up carefully throughout their entire life.

  4. Atypical variants of nodular lymphocyte-predominant Hodgkin lymphoma show low microvessel density and vessels of distention type.

    Science.gov (United States)

    Scheidt, Victoria; Hansmann, Martin-Leo; Schuhmacher, Bianca; Döring, Claudia; Hartmann, Sylvia

    2017-02-01

    Nodular lymphocyte-predominant Hodgkin lymphoma (NLPHL) presents different histopathologic growth patterns, including atypical forms showing overlapping histopathologic and clinical features with T-cell/histiocyte-rich large B-cell lymphoma (THRLBCL). Because growth patterns are associated with vessel distribution, the aim of the present study was to compare angiogenesis in different NLPHL patterns with THRLBCL as well as other lymphomas. Atypical variants of NLPHL and THRLBCL (n=10 per group) both showed a low microvessel density (MVD; 1.16-1.31/μm(2)) with a diffuse vessel distribution. In contrast, in typical NLPHL (n=10), follicular areas with low MVD were retained, whereas an increase in vessels in the interfollicular areas was observed (MVD 1.35/μm(2)). THRLBCL and typical NLPHL could additionally be distinguished by differences in their molecular angiogenesis signature. Furthermore, the number of intravascular T cells was significantly reduced in THRLBCL (0.0028 T cells/mm(2) vessel area) when compared with typical NLPHL (0.0059 T cells/mm(2) vessel area), potentially reflecting the different composition of the microenvironment in these 2 lymphoma entities. The results of our study reveal a similar vascular pattern and angiogenesis behavior in atypical NLPHL variants and THRLBCL in contrast to the retained follicular pattern in typical NLPHL. Copyright © 2016 Elsevier Inc. All rights reserved.

  5. {sup 18F} FDG PET Demonstration of Cancer Recurrence Presenting as Dermatomyositis in a Rare Case of Primary Pleural Lymphoma

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    Lee, Sang Mi; Kim, Yu Kyeong; Lee, Jong Jin; Lee, Yoon Jong; Kim, Hye Ryung; Choe, Gheeyoung [Seoul National Univ. College of Medicine/Bundang Hospital, Seoul (Korea, Republic of); Lee, Jeong Won [Jeju National Univ. Hospital, Jeju (Korea, Republic of)

    2011-03-15

    Dermatomyositis (DM) or polymyositis (PM) are possibly considered to have an association with malignancies. We describe a case of dermatomyositis in which {sup 18F} fluorodeoxyglucose (FDG)positron emission tomography (PET) was able to detect cancer recurrence earlier than any other modality in a patient with a history of primary pleural lymphoma, a very rare condition of malignancy. Further, a typical finding of dermatomyositis is diffuse hypermetabolism in the bilateral proximal shoulder and pelvic girdle areas was shown on {sup 18F} FDG PET, which can implicate the inflammatory process in the skeletal muscle in dermatomyosistis. This case well illustrates the characteristic {sup 18F} FDG findings of dermatomyositis as well as a capability of {sup 18F} FDG PET in detection of recurrence of lymphoma, even in a rare condition.

  6. Bilateral primary breast lymphoma

    Institute of Scientific and Technical Information of China (English)

    Jung Im Yi; Byung Joo Chae; Ja Seong Bae; Bong Joo Kang; Ahwon Lee; Byung Joo Song; Sang Seol Jung

    2010-01-01

    @@ Primary breast lymphoma (PBL) is rare, accounting for 0.04%-0.50% of breast malignancies and 1.7% of extranodal lymphoma.1,2 The originally described diagnostic criteria for PBL2 remains the standard definition for this disease. These criteria are breast location as the clinical site of presentation, absence of history of previous lymphoma or evidence of widespread disease at diagnosis, close association of lymphoma with breast tissue in pathologic specimens, and involvement of ipsilateral lymph nodes if they develop simultaneously with PBL.

  7. Bilateral Primary Intraocular Lymphoma

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    Mehrdad Karimi

    2011-01-01

    Full Text Available Purpose: To report a case of bilateral primary intraocular lymphoma. Case report: A 33-year-old man presented with bilateral blurred vision since two years ago. Examination revealed large keratic precipitates, anterior chamber reaction, posterior subcapsular cataracts, and vitreous infiltration. After a short trial of topical and periocular steroids, diagnostic 25-gauge pars plana vitrectomy was performed and cytologic evaluation of the aspirate confirmed a diagnosis of intraocular lymphoma. The patient was subsequently managed with intravitreal methotrexate in both eyes and responded favorably. Central nervous system workup for lymphoma was negative. Conclusion: Primary intraocular lymphoma should be considered in young adults suffering from chronic recalcitrant panuveitis.

  8. Follicular-patterned thyroid lesions

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    F. Fulya KÖYBAŞIOĞLU

    2009-01-01

    Full Text Available Aim: Our aim is to determine the minimal cytopathologic criteria needed to make differential diagnosis in follicular-patterned lesions of the thyroid gland.Materials and Methods: We reviewed 56 fine needle aspiration cytology specimens which were reported as “suspicious for follicular-patterned lesions of thyroid” between years 2001 and 2005 in our hospital and their histological slides. Parameters for cytopathologic assesment are cellularity, colloid formation, multilayered rosette formation, follicular cell rings, monolayered sheets, intact follicles, hyperplastic papillae, hyaline stromal fragments, intranuclear inclusions, nuclear grooves, angulated nuclei, nucleoli, cerebriform nuclei, nuclear size, macrophages, flame cells and Hurthle cells. Statistical analysis was performed using χ2 and Fisher's-exact tests and Kolmogorov-Simirnov test.Results: Four cytopathologic features–cerebriform nuclei, angulated nuclei, nuclear grooves and intranuclear inclusion- were constantly observed in the follicular variant of papillary carcinoma (p< 0.05. Diluted colloid, monolayered sheet, nuclear size, macrophage and nucleoli were frequently seen in nodular hyperplasia (p< 0.05. The nuclear size was the sole differential cytopathologic criteria between follicular adenoma and follicular carcinoma (p<0.05.Conclusion: Detailed cytopathologic examination was found to be important in differentiating follicular variant of papillary carcinoma from nodular hyperplasia. On the other hand, none of the cytopathologic findings were sufficient to distinguish follicular adenoma from follicular carcinoma. Therefore, cytopathologists should report such lesions as “follicular neoplasms”.

  9. Double- and triple-hit lymphomas can present with features suggestive of immaturity, including TdT expression, and create diagnostic challenges.

    Science.gov (United States)

    Moench, Laura; Sachs, Zohar; Aasen, Garth; Dolan, Michelle; Dayton, Vanessa; Courville, Elizabeth L

    2016-11-01

    Double- and triple-hit lymphomas (DHL/THL) are aggressive B-cell neoplasms characterized by translocation of MYC with concurrent BCL2 and/or BCL6 translocation. In this retrospective study from one institution, we report clinicopathologic features of 13 cases (9 DHL/4 THL). The median age was 59 years (range 30-74) and patients included eight females and five males. Presentation included enlarging lymphadenopathy/masses (11 patients) and abnormal peripheral blood findings (2 patients). Features which raised the differential of an immature neoplasm included terminal deoxynucleotidyl transferase positivity (four cases, two THL/two DHL); dim CD45 expression (seven cases), lack of CD20 (two cases), or lack of surface immunoglobulin light chain (three cases) by flow cytometry; and blastoid morphology (two cases). We conclude that expression of TdT in a B-cell lymphoma with mature features or expression of surface light chain in a case otherwise suggestive of B-lymphoblastic leukemia/lymphoma should prompt an expedited evaluation for DHL/THL.

  10. Pediatric lymphomas in Brazil

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    Gabriela Gualco

    2010-01-01

    Full Text Available OBJECTIVE: This study provides the clinical pathological characteristics of 1301 cases of pediatric/adolescent lymphomas in patients from different geographic regions of Brazil. METHODS: A retrospective analyses of diagnosed pediatric lymphoma cases in a 10-year period was performed. We believe that it represents the largest series of pediatric lymphomas presented from Brazil. RESULTS: Non-Hodgkin lymphomas represented 68% of the cases, including those of precursor (36% and mature (64% cell origin. Mature cell lymphomas comprised 81% of the B-cell phenotype and 19% of the T-cell phenotype. Hodgkin lymphomas represented 32% of all cases, including 87% of the classical type and 13% of nodular lymphocyte predominant type. The geographic distribution showed 38.4% of the cases in the Southeast region, 28.7% in the Northeast, 16.1% in the South, 8.8% in the North, and 8% in the Central-west region. The distribution by age groups was 15-18 years old, 33%; 11-14 years old, 26%; 6-10 years old, 24%; and 6 years old or younger, 17%. Among mature B-cell lymphomas, most of the cases were Burkitt lymphomas (65%, followed by diffuse large B-cell lymphomas (24%. In the mature T-cell group, anaplastic large cell lymphoma, ALK-positive was the most prevalent (57%, followed by peripheral T-cell lymphoma, then not otherwise specified (25%. In the group of classic Hodgkin lymphomas, the main histological subtype was nodular sclerosis (76%. Nodular lymphocyte predominance occurred more frequently than in other series. CONCLUSION: Some of the results found in this study may reflect the heterogeneous socioeconomical status and environmental factors of the Brazilian population in different regions.

  11. Intravascular large B cell lymphoma

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    Ricardo García-Muñoz

    2014-01-01

    Full Text Available Intravascular large B cell lymphoma (IVBCL is a rare type of extranodal large B cell lymphoma characterized by selective growth of lymphoma cells within the microvasculature. We present an illustrative case of intravascular B cell lymphoma suspected by the presence of a very small monoclonal B cell population identified by immunophenotype and polymerase chain reaction in bone marrow. The diagnosis was confirmed by skin biopsy.

  12. Primary cutaneous follicle center lymphoma with Hodgkin and Reed-Sternberg-like cells: a new histopathologic variant.

    Science.gov (United States)

    Dilly, Marie; Marie, Dilly; Ben-Rejeb, Houda; Houda, Ben-Rejeb; Vergier, Béatrice; Béatrice, Vergier; Feldis, Matthieu; Matthieu, Feldis; Toty, Louis; Louis, Toty; Nohra, Olivier; Olivier, Nohra; Beylot-Barry, Marie; Marie, Beylot-Barry; Gros, Audrey; Audrey, Gros; Merlio, Jean-Philippe; Jean-Philippe, Merlio; Parrens, Marie; Marie, Parrens

    2014-10-01

    Primary cutaneous follicle center lymphoma (PCFCL) is the most frequent cutaneous B-cell lymphoma. A 62-year-old man presented with a solitary indolent subcutaneous nodule for 3 years duration, without other abnormalities. Histological examination showed lymphoproliferation with a nodular growth pattern characterized by fibrous collagen bands surrounding nodules. The nodules were composed of medium-sized centrocytes admixed with many large multilobulated and lacunar cells without eosinophils or granulomatous aspect. Hodgkin-like cells were CD30+, CD15+, PAX5+, OCT2+, BOB1+, MUM1+, Ki67+, Bcl6+ and focally CD20+ and EMA-, CD79a-, Bcl2- and CD10-. The medium-sized cells were CD20+, CD79a+, Bcl2+, Bcl6+ and CD10+, enmeshed in a network of CD21-positive follicular dendritic cells. Epstein-Barr virus detection was negative. Interphase fluorescence in situ hybridization showed the absence of BCL2 or BCL6 rearrangement. In such a case, the presence of Hodgkin-like cells intermixed with the tumor population may result in a pitfall diagnosis of classical Hodgkin lymphoma (CHL). Differential diagnoses to be ruled out are secondary or primary skin localization of rather CHL, or systemic follicular lymphoma. Several clinical, radiological, histological, immunohistochemical and molecular arguments indicated the diagnosis of PCFCL. To our knowledge, this is the first report of PCFCL with Hodgkin-like cells. © 2014 John Wiley & Sons A/S. Published by John Wiley & Sons Ltd.

  13. ISOLATION OF CHICKEN FOLLICULAR DENDRITIC CELLS

    Science.gov (United States)

    The aim of the present study was to isolate chicken follicular dendritic cells (FDC). A combination of methods involving panning, iodixanol density gradient centrifugation, and magnetic cell separation technology made it possible to obtain functional FDC from the cecal tonsils from chickens, which h...

  14. Non-Hodgkin lymphoma

    Science.gov (United States)

    Lymphoma - non-Hodgkin; Lymphocytic lymphoma; Histiocytic lymphoma; Lymphoblastic lymphoma; Cancer - non-Hodgkin lymphoma ... National Cancer Institute: PDQ adult non-Hodgkin lymphoma treatment. Bethesda, MD: National Cancer Institute. Updated ... . Accessed ...

  15. [Secondary orbital lymphoma].

    Science.gov (United States)

    Basanta, I; Sevillano, C; Álvarez, M D

    2015-09-01

    A case is presented of an 85 year-old Caucasian female with lymphoma that recurred in the orbit (secondary ocular adnexal lymphoma). The orbital tumour was a diffuse large B-cell lymphoma according to the REAL classification (Revised European-American Lymphoma Classification). Orbital lymphomas are predominantly B-cell proliferations of a variety of histological types, and most are low-grade tumours. Patients are usually middle-aged or elderly, and it is slightly more common in women. A palpable mass, proptosis and blepharoptosis are the most common signs of presentation. Copyright © 2011 Sociedad Española de Oftalmología. Published by Elsevier España, S.L.U. All rights reserved.

  16. Autopsied case of primary malignant lymphoma of the central nervous system presenting an unusual clinical course and CT findings

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    Yamashita, Kazuya; Kobayashi, Shotai; Yamaguchi, Shuhei and others

    1987-08-01

    A case of primary malignant lymphoma of the central nervous system was reported. A 58-year-old man was admitted because of diplopia in March, 1986. Last year in June he lost consciousness, accompanied by headache, vertigo, a floating sensation, and tinnitus, though his symptoms disappeared the next day. Last year in October and November, he complained of weakness of the left hand, but it soon disappeared. A neurological examination on admission revealed left trochlear nerve palsy, a decreased sensitivity to pain on the left side of the face and the opposite side of the body, and a mild left-side lack of coordination. A head CT scan and angiography showed no abnormalities. An examination of the CSF revealed increased protein with mild pleocytosis and IgG, but cytology was negative. After admission, he complained of left trigeminal neuralgia, but it disappeared upon steroid pulse therapy. When the steroids were tapered off, however, peripheral facialnerve palsy developed. Therefore, a second course of steroid pulse therapy was done, with some effect. In June, however, the patient became unconscious while the orally administered steroid was being tapered off. A head CT scan showed isodensity masses in the basal ganglia, the thalamus, and the periventricular white matter on a plain scan, and homogeneous masses with ring enhancement and edema on the use of a contrast medium. A histopathological examination showed primary cerebral malignant lymphoma (large-cell type).

  17. CD20-negative DLBCL transformation after rituximab treatment in follicular small cleaved cell lymphoma-a clinicopathological analysis and review of the literature%滤泡型小裂细胞性淋巴瘤利妥昔单抗治疗后转变为CD20阴性的弥漫大B细胞淋巴瘤临床病理分析并文献复习

    Institute of Scientific and Technical Information of China (English)

    杨翔; 张瑗; 吴宁; 刘瑜; 王璇; 吴楠; 周晓军; 万文辉

    2016-01-01

    -old male who initially presented with enlargement of letf parotid lymph node in 1987. He was diagnosed with follicular small cleaved cell lymphoma through histological evaluation of letf parotid lymph node biopsy in 1988. He was treated with 50 courses of cyclophosphamide, vincristine, prednisone (COP or OP), and local irradiation therapy. In 1998, he developed a high white blood cell count and an increased percentage of lymphocytes. His bone marrow aspiration simultaneously showed chronic lymphocytic leukemia. He received some cycles of rituximab treatment due to a sharp increase in white blood cells from March 2012 to March 2014. He relapsed with a sore throat in March 2014, and the biopsy of right side of epiglottis revealed CD20-negative DLBCL. hTen he received 2 cycles of rituximab combined with low-dose CHOP (mini-CHOP) before the mass in the right side of the epiglottis disappeared. While he relapsed again with the enlargement of right neck lymph node in May 2015, and the lymph node biopsy indicated CD20-positive DLBCL.Conclusion: It is not rare of the loss of CD20 antigen expression in malignant B-cell lymphoma after rituximab treatment. We recommend relapsed or non-sensitive patients atfer therapy with rituximab to rebiopsy for pathological diagnosis, immunohistochemistry, and even molecular genetic testing if necessary. These can reduce misdiagnosis, and play an important role in adjusting therapy of relapsed patients.

  18. Elevation of serum interleukins 8, 4, and 1β levels in patients with gastrointestinal low-grade B-cell lymphoma.

    Science.gov (United States)

    Miyata-Takata, Tomoko; Takata, Katsuyoshi; Toji, Tomohiro; Goto, Naoe; Kasahara, Senji; Takahashi, Takeshi; Tari, Akira; Noujima-Harada, Mai; Miyata, Takafumi; Sato, Yasuharu; Yoshino, Tadashi

    2015-12-17

    Proinflammatory cytokines that are produced by helper T cells (Th) regulate immune reactions, facilitate class switching of B cells, and prolong the lifespan of B and T cells. Eradication therapy using antibiotics is sometimes effective against gastrointestinal (GI) malignant lymphoma, suggesting that the tumor development or progression is affected by the inflammatory microenvironment. In the present study, serum samples from 148 patients with various subtypes of malignant lymphoma were tested for 11 proinflammatory Th1/Th2 cytokines. In the comparison by subtype or GI lesions, serum interleukin (IL)-8 (P = 6.7E-05), IL-4 (P = 7.5E-05), and IL-1β (P = 0.0043) levels showed significant differences among subtypes, being particularly elevated in follicular lymphomas (FL) and mucosa-associated lymphoid tissue (MALT) lymphomas. Serum IL-8 levels were elevated in GI-FL and MALT lymphomas, and serum IL-4 and IL-1 β levels were elevated in MALT lymphomas. These findings show that GI low-grade B-cell lymphoma could develop against the background of an inflammatory microenvironment. Thus, these cytokines may be useful as diagnostic markers and could provide new insights into tumor development.

  19. Large B- Cell lymphoma presenting as acute abdominal pain and spontaneous splenic rupture; A case report and review of relevant literature

    Science.gov (United States)

    Biswas, Saptarshi; Keddington, Judith; McClanathan, James

    2006-01-01

    Background Spontaneous rupture of the spleen is an uncommon dramatic abdominal emergency that requires immediate diagnosis and prompt surgical treatment to ensure the patients survival. Infections have been cited in most cases involving splenic rupture but are rare in hematological malignancies despite frequent involvement of the spleen. Methods and Materials We present a case of a splenic rupture caused by infiltration of B-cell lymphoma. A 43 year old gentleman presented with a 1 day h/o left upper quadrant pain; nausea and vomiting for 2 days with associated dizziness and anorexia. The CT showed abnormal spleen 20 × 11 cm with free fluid in the abdomen and enlarged retroperitoneal LNs. The patient underwent a splenectomy after initial resuscitation and the operative finding was that of a massively enlarged spleen with areas of tumor extruding through the splenic capsule. Result and conclusion Although the spleen is often involved in hematological malignancies, splenic rupture is an infrequent occurrence. In a recent literature review 136 cases were of splenic rupture secondary to hematological malignancy were identified. Acute leukemia and non Hodgkin lymphoma were the frequent causes followed by chronic myelogeneous leukemia. Male sex, adulthood, severe splenomegaly and cytoreductive chemotherapy were factors more often associated with splenic rupture. Emergency splenectomy remains the cornerstone treatment for splenic rupture. We present a case report of a "spontaneous splenic rupture" and discuss the presentation, etiology and treatment options along with discussion of relevant literature PMID:17129392

  20. Sarcoidosis Occurring After Lymphoma

    Science.gov (United States)

    London, Jonathan; Grados, Aurélie; Fermé, Christophe; Charmillon, Alexandre; Maurier, François; Deau, Bénédicte; Crickx, Etienne; Brice, Pauline; Chapelon-Abric, Catherine; Haioun, Corinne; Burroni, Barbara; Alifano, Marco; Le Jeunne, Claire; Guillevin, Loïc; Costedoat-Chalumeau, Nathalie; Schleinitz, Nicolas; Mouthon, Luc; Terrier, Benjamin

    2014-01-01

    Abstract Sarcoidosis is a granulomatous disease that most frequently affects the lungs with pulmonary infiltrates and/or bilateral hilar and mediastinal lymphadenopathy. An association of sarcoidosis and lymphoproliferative disease has previously been reported as the sarcoidosis-lymphoma syndrome. Although this syndrome is characterized by sarcoidosis preceding lymphoma, very few cases of sarcoidosis following lymphoma have been reported. We describe the clinical, biological, and radiological characteristics and outcome of 39 patients presenting with sarcoidosis following lymphoproliferative disease, including 14 previously unreported cases and 25 additional patients, after performing a literature review. Hodgkin lymphoma and non-Hodgkin lymphoma were equally represented. The median delay between lymphoma and sarcoidosis was 18 months. Only 16 patients (41%) required treatment. Sarcoidosis was of mild intensity or self-healing in most cases, and overall clinical response to sarcoidosis was excellent with complete clinical response in 91% of patients. Sarcoidosis was identified after a follow-up computerized tomography scan (CT-scan) or 18fluorodeoxyglucose-positron emission tomography/computerized tomography (18FDG-PET/CT) evaluation in 18/34 patients (53%). Sarcoidosis is therefore a differential diagnosis to consider when lymphoma relapse is suspected on a CT-scan or 18FDG-PET/CT, emphasizing the necessity to rely on histological confirmation of lymphoma relapse. PMID:25380084

  1. Primary extranodal non-Hodgkin's lymphoma of the lung presenting with bilateral, patchy infiltrates dramatically improving after corticosteroid therapy.

    Science.gov (United States)

    Boon, E S; Graal, M B; van Noord, J A

    1993-10-01

    A 63-year-old man was admitted to the hospital with fever and bilateral, peripheral infiltrates. Infectious disease and malignancy seemed to be excluded by fiberoptic diagnostic procedures. Subsequently, respiratory insufficiency developed, making open lung biopsy impossible. The diagnosis of bronchiolitis obliterans organizing pneumonia (BOOP) was strongly considered and treatment with corticosteroids was started; this led to dramatic clinical and radiologic improvement for a short time. Eventually, an open lung biopsy specimen disclosed primary extranodal non-Hodgkin's lymphoma of T-cell origin, immunoblastic, of high-grade malignancy according to the Kiel classification. After the first course of chemotherapy, total respiratory insufficiency developed and the patient died. This case is unique in a patient without AIDS.

  2. Primary anaplastic large cell lymphoma of the breast arising in reconstruction mammoplasty capsule of saline filled breast implant after radical mastectomy for breast cancer: an unusual case presentation

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    Sur Monalisa

    2009-04-01

    Full Text Available Abstract Background Primary non-Hodgkin lymphoma (NHL of the breast represents 0.04–0.5% of malignant lesions of the breast and accounts for 1.7–2.2% of extra-nodal NHL. Most primary cases are of B-cell phenotype and only rare cases are of T-cell phenotype. Anaplastic large cell lymphoma (ALCL is a rare T-cell lymphoma typically seen in children and young adults with the breast being one of the least common locations. There are a total of eleven cases of primary ALCL of the breast described in the literature. Eight of these cases occurred in proximity to breast implants, four in relation to silicone breast implant and three in relation to saline filled breast implant with three out of the eight implant related cases having previous history of breast cancer treated surgically. Adjuvant postoperative chemotherapy is given in only one case. Secondary hematological malignancies after breast cancer chemotherapy have been reported in literature. However in contrast to acute myeloid leukemia (AML, the association between lymphoma and administration of chemotherapy has never been clearly demonstrated. Case Presentation In this report we present a case of primary ALCL of the breast arising in reconstruction mamoplasty capsule of saline filled breast implant after radical mastectomy for infiltrating ductal carcinoma followed by postoperative chemotherapy twelve years ago. Conclusion Primary ALK negative ALCL arising at the site of saline filled breast implant is rare. It is still unclear whether chemotherapy and breast implantation increases risk of secondary hematological malignancies significantly. However, it is important to be aware of these complications and need for careful pathologic examination of tissue removed for implant related complications to make the correct diagnosis for further patient management and treatment. It is important to be aware of this entity at this site as it can be easily misdiagnosed on histologic grounds and to exclude

  3. CXCR5(+)CD8(+) T cells present elevated capacity in mediating cytotoxicity toward autologous tumor cells through interleukin 10 in diffuse large B-cell lymphoma.

    Science.gov (United States)

    Tang, Jiahong; Zha, Jie; Guo, Xutao; Shi, Pengcheng; Xu, Bing

    2017-09-01

    Diffuse large B-cell lymphoma (DLBCL) is a common and aggressive subtype of non-Hodgkin's lymphomas, with limited treatment options in refractory and relapsed patients. Growing evidence supports the notion that CD8(+) T cell immunity could be utilized to eliminate B cell lymphomas. CXCR5(+)CD8(+) T cell is a novel cell subtype and share CXCR5 expression with CD19(+) tumor cells. In this study, we investigated the frequency and function of existing CXCR5(+)CD8(+) T cells in DLBCL patients. We found that DLBCL patients as a group demonstrated significantly higher level of CXCR5(+)CD8(+) T cells than healthy individuals, with huge variability in each patient. Using anti-CD3/CD28-stimulated CD8(+) T cells as effector (E) cells and autologous CD19(+) tumor cells as target (T) cells, at high E:T ratio, no difference between the intensities of CXCR5(+)CD8(+) T cell- and CXCR5(-)CD8(+) T cell-mediated cytotoxicity were observed. However, at intermediate and low E:T ratios, the CXCR5(+)CD8(+) T cells presented stronger cytotoxicity than CXCR5(-)CD8(+) T cells. The expressions of granzyme A, granzyme B, and perforin were significantly higher in CXCR5(+)CD8(+) T cells than in CXCR5(-)CD8(+) T cells, with no significant difference in the level of degranulation. Tumor cells in DLBCL were known to secrete high level of interleukin 10 (IL-10). We therefore blocked the IL-10/IL-10R pathway, and found that the expressions of granzyme A, granzyme B, and perforin by CXCR5(+)CD8(+) T cells were significantly elevated. Together, these results suggest that CXCR5(+)CD8(+) T cells are potential candidates of CD8(+) T cell-based immunotherapies, could mediate elimination of autologous tumor cells in DLBCL patients, but are also susceptible to IL-10-mediated suppression. Copyright © 2017. Published by Elsevier B.V.

  4. Follicular unit extraction as a therapeutic option for Vitiligo

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    S Sacchidanand

    2013-01-01

    Full Text Available Follicular unit extraction (FUE is a surgical procedure, which can be used to transplant follicular units into vitiliginous areas. Such follicular unit transplant has been recently used to repigment stable vitiligo patches. FUE was done for a 12-year-old female with a stable vitiligo patch with leukotrichia on the eyebrow. Repigmentation was noted in 6 weeks and complete pigmentation seen at 12 weeks. Leukotrichia resolved over a period of 6 months. No recurrence was noted at the end of 6 months follow-up with excellent colour match. This case is presented to highlight the simplicity, safety and effectiveness of FUE in stable vitiligo patches with leukotrichia.

  5. Primary pediatric gastrointestinal lymphoma

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    Ranjana Bandyopadhyay

    2011-01-01

    Full Text Available Background: Primary non-Hodgkin′s lymphoma (NHL of the gastrointestinal (GI tract is the most common extranodal lymphoma in pediatric age group. Yet, the overall incidence is very low. The rarity of the disease as well as variable clinical presentation prevents early detection when the possibility of cure exists. Materials and Methods: We studied six cases of primary GI NHL in pediatric age group with reference to their clinical presentation, anatomic distribution and histopathologic characteristics. Results: All were males except one. Intestinal obstruction was the presenting feature in 50%. Half the cases showed ileocaecal involvement, while large bowel was involved in 16%. Histology showed four cases of diffuse large B-cell lymphoma (DLBCL, one case of Burkitt lymphoma, and one Burkitt-like lymphoma. Immunohistochemistry for Tdt, CD20, CD3, CD30, bcl2, bcl6 confirmed the morphological diagnosis. Conclusion: Pediatric GI lymphoma commonly involves the ileocaecal region and presents with intestinal obstruction. A higher prevalence of DLBCL is found compared to other series. A high proliferative index is useful in differentiating Burkitt-like lymphoma from DLBCL.

  6. Familial Follicular-Cell Derived Carcinoma

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    Eun Ju eSon

    2012-05-01

    Full Text Available Follicular cell-derived well-differentiated thyroid cancer, papillary (PTC and follicular thyroid carcinomas (FTC compose 95% of all thyroid malignancies. Familial follicular cell-derived well-differentiated thyroid cancers contribute to 5% of those cases. These familial follicular cell derived carcinomas or non-medullary thyroid carcinomas (NMTC divide into two clinical-pathological groups. One group, syndromic-associated, composed by predominately non-thyroidal tumors, is comprised of Pendred syndrome, Warner syndrome, Carney complex type 1, PTEN-hamartoma tumor syndrome (Cowden disease; PHTS, familial adenomatous polyposis (FAP/Gardner syndrome. Additionally other less established links correlated to the development of follicular cell-derived tumors have also included Ataxia-teleangiectasia syndrome, McCune Albright syndrome, and Peutz-Jeghers syndrome. The subsequent group encompasses syndromes typified by non-medullary thyroid carcinomas or NMTC, as well as, pure familial (f PTC with or without oxyphilia, fPTC with multinodular goiter and fPTC with papillary renal cell carcinoma. This heterogeneous group of diseases has not a established genotype-phenotype correlation as the well-known genetic events identified in the familial C-cell-derived tumors or medullary thyroid carcinomas (MTC. Clinicians should be have the knowledge to identify the likelihood of a patient presenting with thyroid cancer having an additional underlying familial syndrome stemming from characteristics through morphological findings that would alert the pathologist to have the patient undergo subsequent molecular genetics evaluations. This review will discuss the clinical and pathological findings of the patients with familial papillary thyroid carcinoma, such as familial adenomatous polyposis, Carney complex, Werner syndrome, and Pendred syndrome and the heterogeneous group of familial papillary thyroid carcinoma.

  7. Laboratory Treated T Cells in Treating Patients With Relapsed or Refractory Chronic Lymphocytic Leukemia, Non-Hodgkin Lymphoma, or Acute Lymphoblastic Leukemia

    Science.gov (United States)

    2017-07-06

    CD19-Positive Neoplastic Cells Present; Recurrent Adult Acute Lymphoblastic Leukemia; Recurrent Chronic Lymphocytic Leukemia; Recurrent Diffuse Large B-Cell Lymphoma; Recurrent Mantle Cell Lymphoma; Recurrent Non-Hodgkin Lymphoma; Recurrent Small Lymphocytic Lymphoma; Refractory Chronic Lymphocytic Leukemia; Refractory Diffuse Large B-Cell Lymphoma; Refractory Mantle Cell Lymphoma; Refractory Non-Hodgkin Lymphoma; Refractory Small Lymphocytic Lymphoma

  8. Inusual presentación de un Linfoma B cutáneo en un paciente joven Unusual presentation of a cutaneous B Lymphoma in a young male

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    M V Calzinari

    2008-06-01

    Full Text Available Se reporta el caso de un paciente masculino de 23 años de edad, que se presenta con una úlcera de región latero cervical, única, sin compromiso del estado general. Se solicita estudio histopatológico de la lesión e inmunomarcación, con diagnóstico de Linfoma no Hodgkin B de células grandes cutáneos primarios. Se realiza una revisión sobre los Linfomas no Hodgkin B de células grandes cutáneos primarios, siendo anecdótica la presentación en pacientes jóvenes.We report a case of a 23 year old male with a lateral cervical ulcer, without general involvement. Histophatological study and immunomarcation were positive to non Hodgkin Difuse Primary Cutaneous Large B-Cell Lymphoma. A revision was made about Primary Cutaneous B-Cell Lymphomas, being unusual this presentation in young people.

  9. Primary leptomeningeal lymphoma

    Science.gov (United States)

    Taylor, Jennie W.; Flanagan, Eoin P.; O'Neill, Brian P.; Siegal, Tali; Omuro, Antonio; DeAngelis, Lisa; Baehring, Joachim; Nishikawa, Ryo; Pinto, Fernando; Chamberlain, Marc; Hoang-Xuan, Khe; Gonzalez-Aguilar, Alberto; Batchelor, Tracy; Blay, Jean-Yves; Korfel, Agnieszka; Betensky, Rebecca A.; Lopes, Maria-Beatriz S.

    2013-01-01

    Objective: To evaluate clinical presentation, optimal diagnostic evaluation and treatment, and outcome in primary leptomeningeal lymphoma, a rare form of primary CNS lymphoma without parenchymal or systemic involvement. Methods: The International Primary CNS Lymphoma Collaborative Group, a multidisciplinary group of physicians with a particular interest in primary CNS lymphoma, retrospectively identified cases of lymphoma isolated to the leptomeninges as diagnosed by CSF cytology, flow cytometry, or biopsy, without systemic or parenchymal brain/spinal cord lymphoma or immunodeficiency. Results: Forty-eight patients were identified, with median age at diagnosis of 51 years and median Eastern Cooperative Oncology Group performance status of 2. Presenting symptoms were multifocal in 68%. Leptomeningeal enhancement was seen in 74% and CSF profile was abnormal in all cases. CSF cytology detected malignant lymphocytes in 67%. Flow cytometry identified monoclonal population in 80%, as did receptor gene rearrangement studies in 71%. Sixty-two percent had B-cell lymphoma, 19% T-cell, and 19% unclassified. Treatment varied and included fractionated radiotherapy (36%), systemic chemotherapy (78%), and intra-CSF chemotherapy (66%), with 66% receiving ≥2 modalities. Seventy-one percent had a favorable clinical response; ultimately, 44% received salvage treatment. Median overall survival was 24 months, with 11 patients still alive at 50 months follow-up. Conclusion: Primary leptomeningeal lymphoma is a rare form of primary CNS lymphoma. Patients usually present with multifocal symptoms, with evidence of leptomeningeal enhancement and diagnostic CSF analysis. Although treatment is highly variable, patients have a better prognosis than previously reported and a subset may be cured. PMID:24107866

  10. [Application of digital pathology tools. An unusual case of non-Hodgkin lymphoma].

    Science.gov (United States)

    Meyer, A-S K; Dallenbach, F E; Lienert, G; Möller, P; Lennerz, J K

    2012-11-01

    Currently, lymphoma diagnosis is based on a combination of morphology, immunophenotyping, and molecular testing. Using the example of an unusual case of malignant non-Hodgkin lymphoma, we show that improved visualization using digital pathology contributes to the convergence of these complementary diagnostic modalities. A 45-year-old woman presented with skin rash and cervical lymphadenopathy. Histological workup of an excised lymph node showed loss of normal architecture with diffuse infiltration and increased mitotic activity. Immunohistochemistry for CD3/CD5 showed atypical arrangement and infiltration of a T-cell population that dominated over regionally dense, MUM1-positive plasmacellular infiltrates. Expanded CD21/CD23-positive meshworks of follicular dendritic cells were present within and between regressed follicles and the T-cell infiltrate; staining for CD56 and cyclin-D1 was negative. Quantification of Ki-67 staining within the T-, B- and plasmacellular compartments was achieved by digital image conversion, overlay and subsequent quantification algorithms that revealed proliferation within more than 60% of T-cells, over 50% of plasma cells and only 20% of B-cells. Clonality analysis by PCR revealed monoclonal rearrangement for both T-cell receptor gamma chains and immunoglobulin heavy chains. Taken together, we present an unusual combination of an angioimmunoblastic T-cell lymphoma (AITL) and simultaneous plasmacellular lymphoma. This report demonstrates how application of modern tools of digital pathology can visually integrate unusual morphological and molecular findings.

  11. [Histological and immunophenotypical characteristics of peripheral T-cell lymphomas].

    Science.gov (United States)

    Krivolapov, Iu A

    2005-01-01

    Histopathologic features of immunohistochemically confirmed 37 nodal peripheral T-cell lymphomas are described. Unspecified and 10 angioimmunoblastic T-cell lymphomas were analyzed. The most demonstrative histological features of both types of lymphomas were spectrum of small, medium and large lymphoid cells, lymphoid cells with irregular nuclei, presence of clusters of clear cells, arborizing endothelial venules, increased number of histiocytes, eosinophils and plasma cells. Isolated paracortical expantion, compartmentalization of neoplastic infiltrate and large atypical Reed-Stemberg-like cells were occasional findings. Delineation between peripheral T-cell lymphoma, unspecified and angioimmunoblastic T-cell lymphoma needs evaluation of follicular dendritic cell pattern. The results suggest that detection of histopathologic features typical for peripheral T-cell lymphomas gives an opportunity to compose optimal panel for immunotyping which is absolutely necessary.

  12. Hodgkin Lymphoma (For Teens)

    Science.gov (United States)

    ... Can I Help Someone Who's Being Bullied? Volunteering Hodgkin Lymphoma KidsHealth > For Teens > Hodgkin Lymphoma Print A ... to check for disease, including lymphoma. What Is Hodgkin Lymphoma? Hodgkin lymphoma is a type of cancer ...

  13. How I treat double-hit lymphoma.

    Science.gov (United States)

    Friedberg, Jonathan W

    2017-08-03

    The 2016 revision of the World Health Organization (WHO) classification for lymphoma has included a new category of lymphoma, separate from diffuse large B-cell lymphoma, termed high-grade B-cell lymphoma with translocations involving myc and bcl-2 or bcl-6. These lymphomas, which occur in <10% of cases of diffuse large B-cell lymphoma, have been referred to as double-hit lymphomas (or triple-hit lymphomas if all 3 rearrangements are present). It is important to differentiate these lymphomas from the larger group of double-expressor lymphomas, which have increased expression of MYC and BCL-2 and/or BCL-6 by immunohistochemistry, by using variable cutoff percentages to define positivity. Patients with double-hit lymphomas have a poor prognosis when treated with standard chemoimmunotherapy and have increased risk of central nervous system involvement and progression. Double-hit lymphomas may arise as a consequence of the transformation of the underlying indolent lymphoma. There are no published prospective trials in double-hit lymphoma, however retrospective studies strongly suggest that aggressive induction regimens may confer a superior outcome. In this article, I review my approach to the evaluation and treatment of double-hit lymphoma, with an eye toward future clinical trials incorporating rational targeted agents into the therapeutic armamentarium. © 2017 by The American Society of Hematology.

  14. Pain in an osseous lymphoma. A case presentation. Dolor en el linfoma oseo. Presentación de un caso.

    Directory of Open Access Journals (Sweden)

    Alexis Guillén Sánchez

    Full Text Available This is a 79 year-old, white, male patient suffering from osseous lymphoma, a less frequent illness, came to the university Hospital Dr. Gustavo Aldereguía Lima¨ in Cienfuegos city, Cuba complaining of intercostal pain in the left hemithorax. This pain was not relieved with the regular medical treatment, that´s why the anesthesia service doctors gave assistance. Costal osteolysis at level D7, D8 and D9 were shown in a thorax X ray. He was treated with analgesic through peridural via such as morphine, local anesthetic. Betametasone was used as anti-inflammatory having positive results. The use of ethylic alcohol as agents contributed to the patient´s improvement and to get back to his normal life. 

    Se presenta el caso de un paciente del hospital provincial clínico quirúrgico universitario ¨Dr. Gustavo Aldereguía Lima¨ de Cienfuegos, portador de una enfermedad muy poco frecuente (linfoma óseo, de 79 años de edad, masculino, de raza blanca, que refería dolor intercostal en hemitórax izquierdo, el cual no cedía ante el tratamiento médico habitual, por lo que es atendido por anestesiología. En el rayos X de tórax se apreció osteolisis costal a nivel de D7, D8 y D9. En forma frecuente fue tratado con analgésicos por vía peridural: morfina, anestésicos locales y como antiinflamatorio la betametasona con resultados satisfactorios. El uso de agentes como el alcohol etílico, contribuyó a mejorar su cuadro y a que se incorporara a sus labores habituales.

  15. Follicular bronchiolitis: a rare disease in children

    Science.gov (United States)

    Kanık, Esra Toprak; Yılmaz, Özge; Türkeli, Ahmet; Şahin, Şebnem; Yüksel, Hasan

    2014-01-01

    Follicular bronchiolitis (FB) is a benign progressive lung disease. It is characterized with lymphoplasmocellular infiltration and hyperplastic follicles in the peribronchial areas in the small airways. Follicular bronchiolitis should be considered in cases where chronic cough, recurrent upper respiratory tract infections and progressive dyspnea are observed in children. The diagnosis should be supported by lung biopsy. A 8-year old female patient presented to our hospital with complaints including continuing cough and wheezing. Bilateral extensive rales and rhonchi in the lungs were heard on auscultation and lung graphy revealed reticuloglandular appearance. Bilateral extensive septal thickennings, reticulonodular appearance, patchy bronchiectasis, bronchiolectasis and peribronchial thickennings were found on high-resolution thoracal computarized tomography. A diagnosis of follicular bronchiolitis was made as a result of lung biopsy. Improvement was observed in the complaints and findings of our patient after methylprednisolone treatment. This patient was presented to emphasize rare interstitial lung diseases should also be considered in children who present with a clinical picture of chronic bronchial obstruction and do not respond to standard treatment. PMID:26078687

  16. Nivolumab in Treating Patients With HTLV-Associated T-Cell Leukemia/Lymphoma

    Science.gov (United States)

    2017-07-26

    Acute Adult T-Cell Leukemia/Lymphoma; Adult T-Cell Leukemia/Lymphoma; CD3 Positive; CD4-Positive Neoplastic Cells Present; Chronic Adult T-Cell Leukemia/Lymphoma; HTLV-1 Infection; Hypercalcemia; Lymphomatous Adult T-Cell Leukemia/Lymphoma; Recurrent Adult T-Cell Leukemia/Lymphoma; Smoldering Adult T-Cell Leukemia/Lymphoma

  17. The clinical features, management and prognosis of primary and secondary indolent lymphoma of the bone: a retrospective study of the International Extranodal Lymphoma Study Group (IELSG #14 study).

    Science.gov (United States)

    Govi, Silvia; Christie, David; Mappa, Silvia; Marturano, Emerenziana; Bruno-Ventre, Marta; Messina, Carlo; Medina, Elías A Gracia; Porter, David; Radford, John; Heo, Dae Seog; Park, Yeon; Pro, Barbara; Jayamohan, Jayasingham; Pavlakis, Nick; Zucca, Emanuele; Gospodarowicz, Mary; Ferreri, Andrés J M

    2014-08-01

    Indolent lymphomas primarily involving the skeleton (iPBL) represent management and prognosis have not been previously described. Patients with primary and secondary iPBL were selected from an international database of 499 patients with a histopathological diagnosis of non-Hodgkin lymphoma and skeleton involvement, and clinical features, management and prognosis were analyzed. Twenty-six (5%) patients had an iPBL. Ten patients had small lymphocytic lymphoma, 10 had follicular lymphoma and six had lymphoplasmacytic lymphoma. Eleven patients had limited stage and 15 had advanced disease. The overall response rate was 73% (95% confidence interval [CI] = 57-89%). Median follow-up was 58 months, and the 5- and 10-year progression-free survival (PFS) rates were 37 ± 10% and 25 ± 12%, respectively. Nine patients are alive, with 5- and 10-year overall survival (OS) rates of 46 ± 10% and 29 ± 11%, respectively. Patients with small lymphocytic lymphoma showed significantly better outcome than patients with follicular lymphoma. Performance status and stage of disease were independently associated with OS. The prognosis of patients with primary bone lymphoplasmacytic or follicular lymphoma was less favorable.

  18. Linfoma não-Hodgkin apresentando-se como massa hepática única Non-Hodgkin's lymphoma presenting as a single liver mass

    Directory of Open Access Journals (Sweden)

    Mila Correia Góis Peixoto

    2009-02-01

    Full Text Available OBJETIVO: Descrever as principais características de imagem do linfoma não-Hodgkin apresentando-se como massa hepática única. MATERIAIS E MÉTODOS: Realizamos estudo retrospectivo mediante análise de casos de pacientes com massa hepática única aos exames de ultrassonografia, tomografia computadorizada e ressonância magnética, com diagnóstico histológico de linfoma não-Hodgkin. Esses exames foram analisados por dois examinadores em consenso. RESULTADOS: Identificamos três pacientes, todos do sexo masculino, na quinta década de vida, com quadro clínico inespecífico e que apresentavam massa hepática única e com diagnóstico de linfoma não-Hodgkin. Na ultrassonografia a lesão hepática apresentava-se como massa com aspecto "em alvo" nos três casos estudados. Na tomografia computadorizada observou-se massa hipodensa e heterogênea, com realce anelar em todos os casos. Na ressonância magnética as lesões apresentavam-se heterogêneas, hipointensas em T1 e hiperintensas em T2, e também com realce anelar após a injeção do contraste. Nenhum paciente apresentava linfonodomegalia ou comprometimento de outras vísceras sólidas no momento do diagnóstico. CONCLUSÃO: Na presença de massa hepática solitária e com aspecto "em alvo" deve-se considerar, entre as hipóteses, o diagnóstico de linfoma.OBJECTIVE: To describe the main imaging findings of non-Hodgkin's lymphoma presenting as a single liver mass. MATERIALS AND METHODS: A retrospective study was developed with analysis of cases where a single liver mass was observed at ultrasonography, computed tomography and magnetic resonance imaging, and histologically diagnosed as non-Hodgkin's lymphoma. The studies were reviewed by two observers in consensus. RESULTS: Three male patients in the fifth decade of life, with non-specific clinical manifestations and single liver mass diagnosed as non-Hodgkin's lymphoma were identified. A hepatic lesion with target sign was observed at

  19. Hormonal composition of follicular fluid from abnormal follicular structures in mares.

    Science.gov (United States)

    Beltman, M E; Walsh, S W; Canty, M J; Duffy, P; Crowe, M A

    2014-12-01

    The objective was to characterise the hormonal composition of follicular fluid from mares with distinct anovulatory-cystic follicles. Follicular fluid was aspirated from six mares that presented with cystic follicles and from pre-ovulatory follicles of five normal mares (controls). Differences in progesterone, oestradiol, testosterone, IGF-I and IGF binding were analysed using Fisher's exact test. There were greater (P < 0.03) follicular fluid oestradiol concentrations in normal follicles and the testosterone concentration of the cystic fluid was greater (P < 0.05) than that of the normal fluid. There also was a greater (P < 0.03) percentage of IGF-I binding and lower (P < 0.02) IGF-I concentrations in the fluid collected from the cystic structures compared with the fluid from normal follicles. Despite the limited number of animals, the fact that fluid aspirated from cystic follicles had higher testosterone and lower oestradiol concentrations could be of diagnostic value when a practitioner wants to distinguish between a cystic and non-cystic persistent follicle. The research reported here also indicates a likely role for the IGF system in the pathogenesis of the development and maintenance of anovulatory follicular structures in mare ovaries.

  20. Hypothermia & Hodgkin lymphoma in children

    OpenAIRE

    Köse, Doğan; Köksal, Yavuz; Çalışkan, Ümran

    2016-01-01

    Hypothermia associated with Hodgkin lymphoma is defined rarely. This may be caused by a dysfunction that shall occur in hypothalamus, central and peripheral vascular system, skin and muscles. In this study, two Hodgkin lymphoma cases with developed hypothermia are presented. Case 1: An “Hodgkin lymphoma, mixed cellular type” was diagnosed by a biopsy conducted due to lesions found in her spleen on a girl in 7 ages, who applied to the hospital with complaints such as fever, weight loss and nig...

  1. Metastatic thyroid follicular carcinoma of masticator space

    Energy Technology Data Exchange (ETDEWEB)

    Gang, Tae In; Heo, Min Suk; An, Chang Hyeon; Lee, Sam Sun; Choi, Soon Chul; Park, Tae Won [College of Dentistry, Seoul National University, Seoul (Korea, Republic of); Choi, Mi [Department of Dental Radiology, College of Dentistry, Kyungpook National University, Daegu (Korea, Republic of)

    2002-09-15

    Follicular carcinomas are the second most common form of thyroid cancer, accounting for 10 to 20% of all thyroid cancers. Follicular carcinomas have a propensity to metastasize via the bloodstream, spreading to bone, lungs, liver, and elsewhere. We described the case of a 48-year-old woman who presented with swelling of the left pre auricular area, which was a consequence of a metastatic follicular carcinoma of the masticator space. Plain films showed ill defined erosive bony changes from the left condylar head to the mandibular notch. Contrast-enhanced CT images showed a well circumscribed round mass with well enhancement within left masticator space. On MR images, the mass was heterogenously hyperintense to the muscle on T2-weighted images and isointense or hyperintense to the muscle on T1-weighted images, and showed good enhancement on contrast-enhanced T1-weighted images. Upon microscopic examination, the metastatic mass was found to be composed of fairly uniform cells forming small follicles containing colloid, showing capsular and vascular invasion.

  2. Tyrosine phosphorylation in human lymphomas

    NARCIS (Netherlands)

    Haralambieva, E; Jones, M.; Roncador, GM; Cerroni, L; Lamant, L; Ott, G; Rosenwald, A; Sherman, C; Thorner, P; Kusec, R; Wood, KM; Campo, E; Falini, B; Ramsay, A; Marafioti, T; Stein, H; Kluin, PM; Pulford, K; Mason, DY

    2002-01-01

    In a previous study, we showed that the high level of protein tyrosine phosphorylation present in lymphomas containing an anaplastic lymphoma kinase (ALK) can be demonstrated in routinely processed paraffin tissue sections using immunolabelling techniques. In the present study we investigated

  3. Chimeric antigen receptor T-cell therapies for lymphoma.

    Science.gov (United States)

    Brudno, Jennifer N; Kochenderfer, James N

    2017-08-31

    New therapies are needed for patients with Hodgkin or non-Hodgkin lymphomas that are resistant to standard therapies. Indeed, unresponsiveness to standard chemotherapy and relapse after autologous stem-cell transplantation are indicators of an especially poor prognosis. Chimeric antigen receptor (CAR) T cells are emerging as a novel treatment modality for these patients. Clinical trial data have demonstrated the potent activity of anti-CD19 CAR T cells against multiple subtypes of B-cell lymphoma, including diffuse large-B-cell lymphoma (DLBCL), follicular lymphoma, mantle-cell lymphoma, and marginal-zone lymphoma. Importantly, anti-CD19 CAR T cells have impressive activity against chemotherapy-refractory lymphoma, inducing durable complete remissions lasting >2 years in some patients with refractory DLBCL. CAR-T-cell therapies are, however, associated with potentially fatal toxicities, including cytokine-release syndrome and neurological toxicities. CAR T cells with novel target antigens, including CD20, CD22, and κ-light chain for B-cell lymphomas, and CD30 for Hodgkin and T-cell lymphomas, are currently being investigated in clinical trials. Centrally manufactured CAR T cells are also being tested in industry-sponsored multicentre clinical trials, and will probably soon become a standard therapy. Herein, we review the clinical efficacy and toxicity of CAR-T-cell therapies for lymphoma, and discuss their limitations and future directions with regard to toxicity management, CAR designs and CAR-T-cell phenotypes, conditioning regimens, and combination therapies.

  4. The Value of 18F-FDG PET/CT in Diagnostic Procedure of Intravascular Large B-Cell Lymphoma Presenting Fever of Unknown Origin and Pulmonary Hypertension as an Initial Manifestation.

    Science.gov (United States)

    Wu, Fengyu; Wang, Zhenguang; Xing, Xiaoming; Yu, Mingming; Shi, Bin

    2016-06-01

    A 57-year-old man presented with fever of unknown origin and pulmonary hypertension. An F-FDG PET/CT scan was performed to evaluate the source of fever, which showed diffuse, homogeneously increased FDG uptake in both lungs, which prompted the transbronchial lung biopsy. The pathological examination from biopsy specimen demonstrated intravascular large B-cell lymphoma.

  5. Fluorescence immunophenotyping and interphase cytogenetics (FICTION) detects BCL6 abnormalities, including gene amplification, in most cases of nodular lymphocyte-predominant Hodgkin lymphoma.

    Science.gov (United States)

    Bakhirev, Alexei G; Vasef, Mohammad A; Zhang, Qian-Yun; Reichard, Kaaren K; Czuchlewski, David R

    2014-04-01

    BCL6 translocations are a frequent finding in B-cell lymphomas of diverse subtypes, including some cases of nodular lymphocyte-predominant Hodgkin lymphoma (NLPHL). However, reliable analysis of BCL6 rearrangements using fluorescence in situ hybridization is difficult in NLPHL because of the relative paucity of neoplastic cells. Combined immunofluorescence microscopy and fluorescence in situ hybridization, or fluorescence immunophenotyping and interphase cytogenetics as a tool for the investigation of neoplasms (FICTION), permits targeted analysis of neoplastic cells. To better define the spectrum of BCL6 abnormalities in NLPHL using FICTION analysis. We performed an optimized FICTION analysis of 24 lymph nodes, including 11 NLPHL, 5 follicular hyperplasia with prominent progressive transformation of germinal centers, and 8 follicular hyperplasia without progressive transformation of germinal centers. BCL6 rearrangement was identified in 5 of 11 cases of NLPHL (46%). In addition, BCL6 gene amplification, with large clusters of BCL6 signals in the absence of chromosome 3 aneuploidy, was detected in 3 of 11 cases of NLPHL (27%). One NLPHL showed extra copies of BCL6 present in conjunction with multiple copies of chromosome 3. Altogether, we detected BCL6 abnormalities in 9 of 11 cases of NLPHL (82%). None of the progressive transformation of germinal centers or follicular hyperplasia cases showed BCL6 abnormalities by FICTION. To our knowledge, this is the first report of BCL6 gene amplification in NLPHL. Our optimized protocol for FICTION permits detection of cytogenetic abnormalities in most NLPHL cases and may represent a useful ancillary diagnostic technique.

  6. Obinutuzumab for the treatment of indolent lymphoma.

    Science.gov (United States)

    Edelmann, Jennifer; Gribben, John G

    2016-08-01

    Obinutuzumab is a humanized, type II anti-CD20 monoclonal antibody designed for strong induction of direct cell death and antibody-dependent cell-mediated cytotoxicity. The Phase III GADOLIN trial tested the clinical efficacy of obinutuzumab plus bendamustine followed by obinutuzumab monotherapy in rituximab-refractory indolent non-Hodgkin lymphoma versus treatment with bendamustine alone. It demonstrated significantly longer progression-free survival for the obinutuzumab-containing regimen in this difficult to treat patient group. Based on the results of this trial, US FDA approval was most recently granted for obinutuzumab in the treatment of follicular lymphoma that has relapsed after or was refractory to a rituximab-containing regimen. This article summarizes the available data on chemistry, pharmacokinetics, clinical efficacy and safety of obinutuzumab in the treatment of indolent non-Hodgkin lymphoma.

  7. Hodgkin lymphoma - children

    Science.gov (United States)

    Lymphoma - Hodgkin - children; Hodgkin disease - children; Cancer - Hodgkin lymphoma - children; Childhood Hodgkin lymphoma ... In children, Hodgkin lymphoma is more likely to occur between ages 15 to 19 years. The cause of this type of ...

  8. Hodgkin Lymphoma (For Kids)

    Science.gov (United States)

    ... Too Tall or Too Short All About Puberty Hodgkin Lymphoma KidsHealth > For Kids > Hodgkin Lymphoma Print A ... of the cool things he's missed. What Is Hodgkin Lymphoma? Lymphoma (say: lim-FOH-mah) is cancer ...

  9. Occurrence of lymphoma in non-gonadal organ during pregnancy:a report on four cases and literature review

    Institute of Scientific and Technical Information of China (English)

    Da-Lin Gao; Qian-Qian Fu; Tian-Tian Zhang; Lin Sun; Yi Pan; Qiong-Li Zhai

    2016-01-01

    Lymphoma rarely occurs during pregnancy, making this condition difficult to define. Lymphomas that occur in reproductive organs during pregnancy exhibit unique clinical characteristics. Among the limited cases, non-Hodgkin's lymphoma (NHL) shows a considerably higher incidence rate than Hodgkin's lymphoma (HL); NHL also displays clinical characteristics, such as high aggressiveness, advanced stage, and poor outcome. This study reports on four cases of lymphomas in non-gonadal organs (HL, n=2; NHL,n=2) during pregnancy. The tumors rapidly progressed in all patients during pregnancy but remitted at the end of pregnancy and/or therapy. The two HL cases were nodular sclerosis classical HL and treated with chemotherapy after terminating the pregnancy. One of the NHL cases was primary cutaneous follicular center lymphoma, a B cell-derived indolent lymphoma. The patient was followed up without any therapy after terminating her pregnancy. The other case was a follicular lymphoma grade 3B, which was treated with chemotherapy after delivery. We also conducted a literature review of 165 lymphoma cases occurring during pregnancy reported from 1976 to 2013 to reveal the correlation between pregnancy and lymphoma progression. Immunohistochemistry studies were performed to determine the expression of estrogen/progesterone receptors (ER/PR), and ER was weakly positive and sporadic. We concluded that lymphomas occurring during pregnancy should be managed with a prompt and reasonable treatment. High estrogen level in maternal body may affect lymphoma progression.

  10. Actual Incidence and Clinical Behaviour of Follicular Thyroid Carcinoma: An Institutional Experience

    Directory of Open Access Journals (Sweden)

    Carmela De Crea

    2014-01-01

    Full Text Available Follicular thyroid carcinoma classically accounts for 10–32% of thyroid malignancies. We determined the incidence and the behaviour of follicular thyroid carcinoma in an endemic goitre area. A comparative analysis between minimally invasive and widely invasive follicular thyroid carcinoma was performed. The medical records of all patients who underwent thyroidectomy from October 1998 to April 2012 for thyroid malignancies were reviewed. Those who had a histological diagnosis of follicular carcinoma were included. Among 5203 patients, 130 (2.5% were included. Distant metastases at presentation were observed in four patients. Sixty-six patients had a minimally invasive follicular carcinoma and 64 a widely invasive follicular carcinoma. In 63 patients an oxyphilic variant was registered. Minimally/widely invasive ratio was 41/26 for usual follicular carcinoma and 25/38 for oxyphilic variant (P<0.05. Patients with widely invasive tumors had larger tumors (P<0.001 and more frequently oxyphilic variant (P<0.05 than those with minimally invasive tumours. No significant difference was found between widely invasive and minimally invasive tumors and between usual follicular carcinoma and oxyphilic variant regarding the recurrence rate (P=NS. The incidence of follicular thyroid carcinoma is much lower than classically retained. Aggressive treatment, including total thyroidectomy and radioiodine ablation, should be proposed to all patients.

  11. Nivolumab With or Without Varlilumab in Treating Patients With Relapsed or Refractory Aggressive B-cell Lymphomas

    Science.gov (United States)

    2017-03-13

    Activated B-Cell-Like Diffuse Large B-Cell Lymphoma; ALK-Positive Large B-Cell Lymphoma; Atypical Burkitt/Burkitt-Like Lymphoma; Diffuse Large B-Cell Lymphoma Associated With Chronic Inflammation; Diffuse Large B-Cell Lymphoma, Not Otherwise Specified; Epstein-Barr Virus Positive Diffuse Large B-Cell Lymphoma of the Elderly; Epstein-Barr Virus-Positive Mucocutaneous Ulcer; Germinal Center B-Cell-Like Diffuse Large B-Cell Lymphoma; High-Grade B-Cell Lymphoma With MYC and BCL2 and/or BCL6 Rearrangements; Human Herpesvirus-8-Positive Neoplastic Cells Present; Intravascular Large B-Cell Lymphoma; MYC-Negative B-Cell Lymphoma With 11q Aberration Resembling Burkitt Lymphoma; Plasmablastic Lymphoma; Primary Cutaneous Diffuse Large B-Cell Lymphoma; Primary Cutaneous Diffuse Large B-Cell Lymphoma, Leg Type; Primary Diffuse Large B-Cell Lymphoma of the Central Nervous System; Primary Effusion Lymphoma; Recurrent Adult Burkitt Lymphoma; Recurrent Diffuse Large B-Cell Lymphoma; Recurrent Lymphomatoid Granulomatosis; Recurrent Mediastinal (Thymic) Large B-Cell Cell Lymphoma; Refractory Burkitt Lymphoma; Refractory Diffuse Large B-Cell Lymphoma; Refractory Mediastinal (Thymic) Large B-Cell Cell Lymphoma; Skin Ulcer; Small Intestinal B-Cell Lymphoma, Unclassifiable, With Features Intermediate Between Diffuse Large B-Cell Lymphoma and Burkitt Lymphoma; T-Cell/Histiocyte-Rich Large B-Cell Lymphoma

  12. [Femoral osteolytic lesions with soft tissue tumors and hypercalcemia as presentation form of a B-cell lymphoma].

    Science.gov (United States)

    Hernández Hernández, J L; Olmos Martínez, J M; Figols Ladrón de Guevara, J; Riancho Moral, J A; González Macías, J

    2000-05-01

    Hypercalcemia associated with haematological neoplasms account for 15 to 20% of hipercalcemia in malignancy, and occurs usually in patients with multiple myeloma. However, its incidence in patients with linfoma is low, and it is observed usually in T-cell linfomas. Bone affectation is also uncommon in patients with non-Hodgkin linfoma. It usually is seen as a late manifestation of the disease, and its occurrence as the form of presentation is exceptional. We hereby report a patient with a B-cell non-Hodgkin linfoma presenting with hypercalcemia and femoral osteolytic lesions.

  13. Breast lymphoma

    African Journals Online (AJOL)

    Expression of oestrogen receptor protein as determined by ... lymphomas. While this classification has been fairly widely accepted, a ... minimum a full history and physical examination, chest radiographs ... and hepatic function. A number ...

  14. Hodgkin's Lymphoma

    Science.gov (United States)

    ... for information in your local library and on the Internet. Start your information search with the National Cancer ... www.mayoclinic.org/diseases-conditions/hodgkins-lymphoma/basics/definition/CON-20030667 . Mayo Clinic Footer Legal Conditions and ...

  15. Primary lymphoma of the brain

    Science.gov (United States)

    Brain lymphoma; Cerebral lymphoma; Primary lymphoma of the central nervous system; Lymphoma - brain ... The cause of primary brain lymphoma is not known. People with a weakened immune system are at high risk for primary lymphoma of the brain. ...

  16. Laryngeal lymphoma: the high and low grades of rare lymphoma involvement sites.

    Science.gov (United States)

    Azzopardi, Charles Paul; Degaetano, James; Betts, Alexandra; Farrugia, Eric; Magri, Claude; Refalo, Nicholas; Gatt, Alexander; Camilleri, David J

    2014-01-01

    The larynx is an extremely rare site of involvement by lymphomatous disease. We present two cases of isolated laryngeal high-grade and another low-grade lymphoma, together with a literature review of laryngeal lymphoma management.

  17. T-Cell Lymphoma

    Science.gov (United States)

    Getting the Facts T-Cell Lymphoma Overview Lymphoma is the most common blood cancer. The two main forms of lymphoma are Hodgkin lymphoma ... develop into lymphomas: B-lymphocytes (B-cells) and T-lymphocytes (T-cells). T-cell lymphomas account for ...

  18. Expression of DNA mismatch repair proteins in transformed non-Hodgkin's lymphoma: relationship to smoking

    DEFF Research Database (Denmark)

    Nandi, S; Yu, J; Reinert, Line

    2006-01-01

    It has been hypothesized that defects in DNA-mismatch repair are associated with smoking in certain types of transformed non-Hodgkin lymphoma (NHL). We have analyzed biopsy samples from two indolent B-cell lymphomas, follicular lymphoma (FL) and chronic lymphocytic leukemia/small lymphocytic...... leukemia (CLL/SLL), that have transformed to diffuse-large B-cell lymphoma (DLBCL). We correlated the presence or absence of DNA-mismatch repair enzymes by immunostaining as well as the p53 status to smoking history. Of all patients (n = 30), 37% showed negative immunostaining of MLH1, 16% showed negative...

  19. Frequent mutation of histone-modifying genes in non-Hodgkin lymphoma | Office of Cancer Genomics

    Science.gov (United States)

    In a recent Nature article, Morin et al. uncovered a novel role for chromatin modification in driving the progression of two non-Hodgkin lymphomas (NHLs), follicular lymphoma and diffuse large B-cell lymphoma. Through DNA and RNA sequencing of 117 tumor samples and 10 assorted cell lines, the authors identified and validated 109 genes with multiple mutations in these B-cell NHLs. Of the 109 genes, several genes not previously linked to lymphoma demonstrated positive selection for mutation including two genes involved in histone modification, MLL2 and MEF2B.

  20. Prognostic Value of Maximum Standard Uptake on Pretreatment 18F-FDG PET/CT Scan in Newly Diagnosed Follicular Lymphoma%治疗前18F-FDG PET/CT最大标准摄取值在滤泡性淋巴瘤中的预后价值

    Institute of Scientific and Technical Information of China (English)

    丁重阳; 孙晋; 李天女; 徐卫

    2015-01-01

    目的:探讨滤泡性淋巴瘤(FL)患者治疗前1 8 F-FDG PET/CT最大标准摄取值(SUVmax)的预后价值.方法:回顾性分析2005年11月至2013年10月在我院经病理确诊的30例FL患者的病例资料及所有患者于治疗前行18F-FDG PET/CT检查结果,并从病理分级、国际预后指数2的分级、有无肿块及骨髓受累,临床检测指标及治疗转归方面评估,治疗前SUVmax与预后因子、近期疗效及远期生存之间的关系.结果:组织学分为1级、2级和3级患者间的SUVmax差异有统计学意义(P=0.040);滤泡性淋巴瘤国际预后指数2(follicular lymphoma international prognostic index,FLIPI2)低危组、中危组和高危组患者的SUV max差异无统计学意义(P =0.431).有、无大肿块及有、无骨髓受累患者的SUVmax差异均无统计学意义(均P >0.05);SUVmax与分期、β2微球蛋白、血红蛋白含量、LDH及Ki-67均无相关性(均P>0.05).完全缓解与未完全缓解患者的SUVmax、有效患者(完全缓解+部分缓解)与无效患者(疾病稳定+疾病进展)的SUVmax差异均无统计学意义(均P>0.05).分别设定10、15为SUVmax界限值,SUVmax低者与高者的完全缓解率、总有效率、3年PFS率及3年OS率均无统计学意义(均P>0.05).结论:FL患者治疗前18 F-FDG PET/CT的SUVmax预测预后的价值在本组研究中不明确,目前尚不能通过其来判断患者预后.

  1. Cutaneous natural killer/T-cell lymphoma.

    Science.gov (United States)

    Radonich, Michael A; Lazova, Rossitza; Bolognia, Jean

    2002-03-01

    Lymphomas are classified as either Hodgkin's or non-Hodgkin's. The 2 subtypes of non-Hodgkin's lymphoma that can present primarily in the skin are cutaneous T-cell lymphoma and cutaneous B-cell lymphoma, both of which tend to be low-grade malignant neoplasms. Recently another distinct subtype of lymphoma was discovered, the natural killer (NK)/T-cell lymphoma, which can involve the skin in a primary or secondary fashion. The NK/T-cell subtype of lymphoma is characterized by the expression of the NK-cell antigen CD56. These CD56(+) lymphomas are further subdivided into nasal NK/T-cell lymphomas that commonly present as midfacial destructive disease and non-nasal NK/T-cell lymphomas that often arise in extranodal locations, including the skin. We report a case of aggressive NK-cell leukemia/lymphoma with numerous secondary cutaneous lesions and review the clinical and histopathologic spectrum of non-nasal CD56(+) lymphomas, with an emphasis on the dermatologic findings.

  2. Imaging Findings of Follicular Dendritic Cell Sarcoma: Report of Four Cases

    Energy Technology Data Exchange (ETDEWEB)

    Long-Hua, Qiu; Xiao-Yuan, Feng [Affi liated HuaShan Hospital, Fudan University, Shanghai (China); Qin, Xiao; Ya-Jia, Gu; Jian, Wang [Affiliated Cancer Hospital, Fudan University, Shanghai (China)

    2011-02-15

    Follicular dendritic cell sarcoma is a rare malignant neoplasm and little is known about its radiological features. We present here four cases of follicular dendritic cell sarcomas and we provide the image characteristics of these tumors to help radiologists recognize this entity when making a diagnosis

  3. A possible coincidence of cytomegalovirus retinitis and intraocular lymphoma in a patient with systemic non-Hodgkin’s lymphoma

    Directory of Open Access Journals (Sweden)

    Svozílková Petra

    2013-01-01

    Full Text Available Abstract Purpose To present a possible coincidence of cytomegalovirus retinitis and intraocular lymphoma in a patient with systemic non-Hodgkin’s lymphoma. Case presentation A 47-year-old woman presented with decreased visual acuity associated with white retinal lesions in both eyes. A history of pneumonia of unknown aetiology closely preceded the deterioration of vision. Five years previously the patient was diagnosed with follicular non-Hodgkin’s lymphoma. She was treated with a chemotherapy regimen comprised of cyclophosphamide, adriamycin, vincristin, and prednisone with later addition of the anti-CD20 antibody rituximab. She experienced a relapse 19 months later with involvement of the retroperitoneal lymph nodes, and commenced treatment with rituximab and 90Y-ibritumomab tiuxetan. A second relapse occurred 22 months after radioimmunotherapy and was treated with a combination of fludarabine, cyclophosphamide, and mitoxantrone followed by rituximab. The patient experienced no further relapses until the current presentation (April, 2010. Pars plana vitrectomy with vitreous fluid analysis was performed in the right eye. PCR testing confirmed the presence of cytomegalovirus in the vitreous. Atypical lymphoid elements, highly suspicious of malignancy were also found on cytologic examination. Intravenous foscarnet was administered continually for three weeks, followed by oral valganciclovir given in a dose of 900 mg twice per day. In addition, the rituximab therapy continued at three monthly intervals. Nevertheless, cessation of foscarnet therapy was followed by a recurrence of retinitis on three separate occasions during a 3-month period instigating its reinduction to the treatment regime after each recurrence. Conclusions Cytomegalovirus retinitis is an opportunistic infection found in AIDS patients as well as in bone marrow and solid organ transplant recipients being treated with systemic immunosuppressive drugs. This case presents a less

  4. Primary extranodal marginal zone lymphoma of the uvea associated with massive diffuse epibulbar extension and focal infiltration of the optic nerve and meninges, clinically presented as uveitis masquerade syndrome: a case report.

    Science.gov (United States)

    Rasić, D M; Stanković, Z; Terzić, T; Kovacević, D; Koturović, Z; Marković, V

    2010-09-01

    To report a clinical, histopathological and immunohistochemical findings in a case of primary extranodal marginal zone lymphoma of the uvea associated with massive diffuse extraocular episcleral extension and focal infiltration of the optic nerve and meninges, clinically presented as longstanding uveitis masquerade syndrome. Interventional case reports with histopathological correlation. We describe a 80-year-old male patient with a 3-year history of chronic recurrent hypertensive (pan) uveitis associated with ocular pain, unresponsive to topical and systemic anti-inflammatory, immunosuppressive, antibiotic/antiviral and antiglaucomatous therapy. Because the eye was not salvageable with conservative treatment, enucleation of blind and painful eye was performed. Findings from histopathological and immunohistochemistry examination of the enucleated eye showed an extranodal marginal zone lymphoma of the uveal tract with massive epibulbar extension and optic nerve and meningeal penetration. During almost 3 years of clinical course and 6 months after the enucleation, there were no systemic manifestations of lymphoma, and patient has not required subsequent treatment. Primary lymphoproliferative lesions of the uvea, comprising the iris, ciliary body and choroid are very rare, associated with epibulbar extension extremely and with optic nerve and menigeal penetration exceptionally. Despite its rarity, primary lymphoma of the uvea should be included in the differential diagnosis particularly in older patients with longstanding recurrent uveitis.

  5. Lymphoma of the Urinary Bladder

    Directory of Open Access Journals (Sweden)

    Anthony Kodzo-Grey Venyo

    2014-01-01

    Full Text Available Background. Lymphoma of the urinary bladder (LUB is rare. Aims. To review the literature on LUB. Methods. Various internet databases were used. Results. LUB can be either primary or secondary. The tumour has female predominance; most cases occur in middle-age women. Secondary LUB occurs in 10% to 25% of leukemias/lymphomas and in advanced-stage systemic lymphoma. Less than 100 cases have been reported. MALT typically affects adults older than 60 years; 75% are female. Diffuse large B-cell lymphoma is also common and may arise from transformation of MALT. LUB presents with haematuria, dysuria, urinary frequency, nocturia, and abdominal or back pain. Macroscopic examination of LUBs show large discrete tumours centred in the dome or lateral walls of the bladder. Positive staining of LUB varies by the subtype of lymphoma; B-cell lymphomas are CD20 positive. MALT lymphoma is positively stained for CD20, CD19, and FMC7 and negatively stained for CD5, CD10, and CD11c. LUB stains negatively with Pan-keratin, vimentin, CK20, and CK7. MALT lymphoma exhibits t(11; 18(q21: 21. Radiotherapy is an effective treatment for the MALT type of LUB with no recurrence. Conclusions. LUB is diagnosed by its characteristic morphology and immunohistochemical characteristics. Radiotherapy is a useful treatment.

  6. T follicular regulatory cells in mice and men.

    Science.gov (United States)

    Maceiras, Ana Raquel; Fonseca, Valter R; Agua-Doce, Ana; Graca, Luis

    2017-09-01

    It has long been known that CD4 T cells are necessary to provide help to B cells, triggering a germinal centre (GC) reaction where affinity maturation and isotype switching occur. However, the nature of the dedicated CD4 helper T cells, known as T follicular helper (Tfh), was only recently described. Here, we review the biology and function of the recently described T follicular regulatory (Tfr) cells, another CD4 T-cell population also found within GCs but with regulatory function and characteristics. Tfr cells have been identified in mice and humans as simultaneously presenting characteristics of T follicular cells (namely CXCR5 expression) and regulatory T cells (including Foxp3 expression). These Tfr cells have been implicated in the regulation of the magnitude of the GC reaction, as well as in protection from immune-mediated pathology. © 2017 John Wiley & Sons Ltd.

  7. Lymphoma of the Cervix

    Directory of Open Access Journals (Sweden)

    Juanita Parnis

    2012-01-01

    Full Text Available Primary non-Hodgkins lymphoma of the uterine cervix is a very rare diagnosis. A 54-year-old woman presented with a 3-month history of postmenopausal bleeding per vaginum. On examination, a friable, fungating lesion was seen on the cervix. Histology revealed a CD 20 positive high-grade non-Hodgkin’s diffuse large B cell lymphoma from cervical biopsies and endometrial curettage. She was diagnosed as stage IE after workup and subsequently treated with six cycles of R-CHOP chemotherapy followed by radiotherapy of the involved field.

  8. Primary Pancreatic Lymphomas

    Directory of Open Access Journals (Sweden)

    Muhammad Wasif Saif

    2006-05-01

    Full Text Available Extranodal non-Hodgkin’s lymphomas (NHLs represent up to 30-40% of all NHL cases. The gastrointestinal tract is the most commonly involved extranodal site; accounting for about half of such cases [1]. Stomach and the small intestine constitute the most common gastrointestinal sites. Secondary invasion of the pancreas from contiguous, retroperitoneal lymph node disease is the prevalent mode of involvement. Secondary involvement of the pancreas from the duodenum or adjacent peripancreatic lymphadenopathy is well-known. Primary pancreatic lymphoma (PPL is an extremely rare disease [2]. PPL can present as an isolated mass mimicking pancreatic carcinoma. However, unlike carcinomas, PPL are potentially treatable [3].

  9. Primary marginal zone B-cell lymphoma of appendix

    Directory of Open Access Journals (Sweden)

    Radha S

    2008-07-01

    Full Text Available Primary lymphomas of appendix are extremely rare tumors. The first case of primary lymphoma of appendix was reported by Warren in the year 1898. Incidence of primary lymphoma of appendix is 0.015% of all gastrointestinal lymphomas. This is a report of primary marginal zone B-cell lymphoma of appendix which presented as appendicular mass. As some cases are incidentally discovered, this case emphasizes that histological examination of all appendicectomy specimens is mandatory.

  10. Composite ALK-negative anaplastic large cell lymphoma and small lymphocytic lymphoma involving the right inguinal lymph node.

    Science.gov (United States)

    Persad, Paul; Pang, Changlee S

    2014-02-01

    Anaplastic large cell lymphoma and small lymphocytic lymphoma are two lymphoid malignancies with completely distinct morphologies and natural histories. We present a rare case of composite anaplastic large cell lymphoma and small lymphocytic lymphoma in an inguinal lymph node of an otherwise healthy 47-year-old male patient. Immunohistochemical and molecular studies identified the two populations clearly. Their separation is imperative as anaplastic large cell lymphoma can be an aggressive neoplasm and easily overlooked in cases of small lymphocytic lymphoma with a small population of anaplastic large cell lymphoma cells.

  11. High-Dose Busulfan and High-Dose Cyclophosphamide Followed By Donor Bone Marrow Transplant in Treating Patients With Leukemia, Myelodysplastic Syndrome, Multiple Myeloma, or Recurrent Hodgkin or Non-Hodgkin Lymphoma

    Science.gov (United States)

    2010-08-05

    Adult Acute Myeloid Leukemia; Recurrent Adult Burkitt Lymphoma; Recurrent Adult Diffuse Large Cell Lymphoma; Recurrent Adult Diffuse Mixed Cell Lymphoma; Recurrent Adult Diffuse Small Cleaved Cell Lymphoma; Recurrent Adult Grade III Lymphomatoid Granulomatosis; Recurrent Adult Hodgkin Lymphoma; Recurrent Adult Immunoblastic Large Cell Lymphoma; Recurrent Adult Lymphoblastic Lymphoma; Recurrent Adult Non-Hodgkin Lymphoma; Recurrent Adult T-cell Leukemia/Lymphoma; Recurrent Childhood Acute Lymphoblastic Leukemia; Recurrent Childhood Acute Myeloid Leukemia; Recurrent Cutaneous T-cell Non-Hodgkin Lymphoma; Recurrent Grade 1 Follicular Lymphoma; Recurrent Grade 2 Follicular Lymphoma; Recurrent Grade 3 Follicular Lymphoma; Recurrent Mantle Cell Lymphoma; Recurrent Marginal Zone Lymphoma; Recurrent Mycosis Fungoides/Sezary Syndrome; Recurrent Small Lymphocytic Lymphoma; Refractory Multiple Myeloma; Relapsing Chronic Myelogenous Leukemia; Secondary Myelodysplastic Syndromes; Small Intestine Lymphoma; Splenic Marginal Zone Lymphoma; Testicular Lymphoma; Waldenstrom Macroglobulinemia

  12. Novel murine B-cell lymphoma/leukemia model to study BCL2-driven oncogenesis.

    Science.gov (United States)

    Meijerink, Jules P P; Van Lieshout, Esther M M; Beverloo, H Berna; Van Drunen, Ellen; Mensink, Ewald J B M; Macville, Merryn; Pieters, Rob

    2005-05-10

    The BCL-2 family has been implicated in the pathogenesis of various hematopoietic malignancies, including follicular non-Hodgkin lymphoma and B-cell chronic lymphocytic leukemia. To identify genes that act synergistically in BCL2-enforced leukemogenesis, we developed a murine B-cell lymphoma/leukemia model based on the IL-3-dependent Balb/C pro-B line (FL5.12). FL5.12 cells were stably transfected with antiapoptotic BCL-2 alone or in combination with proapoptotic BAX or nonfunctional mutant BAX, thereby creating various levels of imbalance within the BCL-2 family. Transfectants were intravenously injected into normal Balb/C mice. Whereas FL5.12 cells did not provoke leukemia, mice injected with stable transfectants died of leukemia over time. Disease incidence and latency time depended on the degree of imbalance in the BCL-2 family, supporting a model whereby BCL2 drives tumorigenesis. All mice presented with hepatosplenomegaly and leukemic FL5.12 cells in peripheral blood and bone marrow compartments. Leukemic conversion was accompanied by secondary genetic aberrations leading to clonal IL-3-responsive leukemia. Cellular transformation was independent of alterations in c-Myc or downstream apoptotic pathway. Leukemic clones retained a normal DNA damage response leading to elevated P53 and P21 levels and cell cycle arrest upon irradiation. In conclusion, our mouse model may prove a valuable tool to identify genes that cooperate in BCL2-enforced lymphoma/leukemogenesis.

  13. Molecular signatures of thyroid follicular neoplasia

    DEFF Research Database (Denmark)

    Borup, R.; Rossing, M.; Henao, Ricardo

    2010-01-01

    The molecular pathways leading to thyroid follicular neoplasia are incompletely understood, and the diagnosis of follicular tumors is a clinical challenge. To provide leads to the pathogenesis and diagnosis of the tumors, we examined the global transcriptome signatures of follicular thyroid...... a mechanism for cancer progression, which is why we exploited the results in order to generate a molecular classifier that could identify 95% of all carcinomas. Validation employing public domain and cross-platform data demonstrated that the signature was robust and could diagnose follicular nodules...... and robust genetic signature for the diagnosis of FA and FC. Endocrine-Related Cancer (2010) 17 691-708...

  14. Clinical value of 18F-FDG PET-CT to patients with follicular lymphoma staging Ⅲ-Ⅳ: monitoring response to therapy%18F-FDG PET-CT在Ⅲ~Ⅳ期滤泡性淋巴瘤疗效监测中的临床价值

    Institute of Scientific and Technical Information of China (English)

    丁重阳; 李天女; 杨文平; 孙晋; 黄庆娟; 丁其勇; 杨伟

    2013-01-01

    目的 探讨18F-FDG PET-CT对Ⅲ~Ⅳ期滤泡性淋巴瘤(FL)疗效监测的临床价值.方法 回顾性分析20例FL患者资料,所有患者在化疗前、化疗中及化疗后均行18F-FDG PET-CT检查,采用Kaplan-Meier方法对患者进行生存分析.结果 20例患者化疗中期PET-CT检查示14例完全缓解(CR),继续原方案化疗,化疗后PET-CT检查示患者均仍为CR,随访均未见复发.化疗中期PET-CT检查示2例部分缓解(PR),按原方案继续化疗,化疗后1例CR,1例进展(PD),随访均复发.化疗中期PET-CT检查示4例PD,均换化疗方案,化疗后2例CR,1例PR,1例PD,随访发现1例死亡,2例复发,1例CR.中位随访29个月,化疗中期PET-CT阴性预测值为100%(14/14),化疗中期PET-CT检查阳性组与阴性组无进展生存(PFS)比较,差异无统计学意义(24个月比32个月,P=0.135).化疗后PET-CT阴性预测值为88.2%(15/17),化疗后PET-CT阳性组与阴性组PFS比较,差异有统计学意义(8个月比34个月,P=0.0006).结论 18F-FDG PET-CT能有效地监测Ⅲ~Ⅳ期FL化疗疗效,并有助于临床制定个体化治疗方案.%Objective To evaluate the clinical value of 18F-FDG PET-CT in monitoring response to therapy in patients with follicular lymphoma (FL) staging Ⅲ-Ⅳ.Methods The clinical data of 20 patients with FL were analyzed retrospectively.All patients underwent PET-CT at three times:baseline,after 3-4 cycles of chemotherapy,and end of treatment.Kaplan-Meier method was used to analyze the survival of patients.Results 14 patients achieved CR at the interim PET-CT,all patients achieved CR at post therapy PET-CT:all patients were alive with no evidence of disease in the follow-up.2 patiens achieved PR at the interim PET-CT,and 1 patient achieved CR,and 1 patient was PD,at post therapy PET-CT,all patients were relapsed in the follow-up.4 patients achieved PD,and all patients were changed the chemotherapy plan.At post therapy PET-CT,2 patients achieved CR,1 patient achieved PR,and 1

  15. 18F-FDG PET/CT在滤泡性淋巴瘤分期及疗效评价中的临床价值%Clinical value of 18F-FDG PET/CT in clinical staging and therapeutic evaluation of follicular lymphoma

    Institute of Scientific and Technical Information of China (English)

    丁重阳; 李天女; 孙晋; 杨文平; 黄庆娟; 丁其勇; 徐绪党

    2014-01-01

    目的 探讨18F-FDG PET/CT在滤泡性淋巴瘤(FL)的分期、疗效评价、复发监测及预后判断方面的临床价值.方法 回顾性分析2005年12月至2013年1月行PET/CT检查的经病理确诊为FL的28例患者[男12例,女16例,平均年龄57(36~82)岁]资料.对患者均进行Ann Arbor临床分期,统计行PET/CT检查后临床分期改变情况.比较不同临床分期组间、病理高级别(3a+3b级)组和低级别(1+2级)组间SUVmax差异.28例中有17例行化疗后PET/CT检查和电话随访(10~88个月)监测疗效,比较疗效佳(CR+PR)和不佳(SD+PD)者生存差异.统计分析采用Mann-Whitney u秩和检验、Wilcoxon符号秩检验和Kaplan-Meier生存分析.结果 (1)28例治疗前行18F-FDG PET/CT的患者中,10.7% (3/28)分期上调,3.6% (1/28)分期下调.Ⅰ+Ⅱ期组SUVmax为10.1±3.2,Ⅲ+Ⅳ期组SUVmax为11.5±4.9,差异无统计学意义(Z=-0.619,P>0.05).病理低级别组(15例)和高级别组(13例)SUVmax分别为6.9±3.6和12.4±5.6(Z=-3.706,P<0.01).(2)17例治疗前后均行18F-FDG PET/CT检查的患者中,疗效佳组(11例)治疗前SUVmax10.8±5.1,治疗后SUVmax 3.4±2.3(Z=-2.312,P<0.05);疗效不佳组(6例)治疗前SUVmax 11.2±6.9,治疗后SUVmax 7.8±3.3,差异无统计学意义(Z=-1.153,P>0.05).疗效佳与疗效不佳组的中位无进展生存期(PFS)分别为48和26个月(x2=4.207,P<0.05).结论 18F-FDG PET/CT有助于明确FL分期、评价疗效、监测复发及提示预后.%Objective To investigate the clinical value of 18F-FDG PET/CT in staging,therapeutic response evaluation,relapse early detection and prognostic prediction of follicular lymphoma (FL).Methods Twenty-eight patients (12 males,16 females; average age 57 (36-82) years) with pathologically confirmed FL from December 2005 to January 2013 were enrolled.All patients underwent 18F-FDG PET/CT before treatment.The SUVmax of different staging groups,different pathological grade groups (high:3a+3b; low:1+2) was compared

  16. Identification of highly methylated genes across various types of B-cell non-hodgkin lymphoma.

    Directory of Open Access Journals (Sweden)

    Nicole Bethge

    Full Text Available Epigenetic alterations of gene expression are important in the development of cancer. In this study, we identified genes which are epigenetically altered in major lymphoma types. We used DNA microarray technology to assess changes in gene expression after treatment of 11 lymphoma cell lines with epigenetic drugs. We identified 233 genes with upregulated expression in treated cell lines and with downregulated expression in B-cell lymphoma patient samples (n = 480 when compared to normal B cells (n = 5. The top 30 genes were further analyzed by methylation specific PCR (MSP in 18 lymphoma cell lines. Seven of the genes were methylated in more than 70% of the cell lines and were further subjected to quantitative MSP in 37 B-cell lymphoma patient samples (diffuse large B-cell lymphoma (activated B-cell like and germinal center B-cell like subtypes, follicular lymphoma and Burkitt`s lymphoma and normal B lymphocytes from 10 healthy donors. The promoters of DSP, FZD8, KCNH2, and PPP1R14A were methylated in 28%, 67%, 22%, and 78% of the 36 tumor samples, respectively, but not in control samples. Validation using a second series of healthy donor controls (n = 42; normal B cells, peripheral blood mononuclear cells, bone marrow, tonsils and follicular hyperplasia and fresh-frozen lymphoma biopsies (n = 25, confirmed the results. The DNA methylation biomarker panel consisting of DSP, FZD8, KCNH2, and PPP1R14A was positive in 89% (54/61 of all lymphomas. Receiver operating characteristic analysis to determine the discriminative power between lymphoma and healthy control samples showed a c-statistic of 0.96, indicating a possible role for the biomarker panel in monitoring of lymphoma patients.

  17. Hodgkin Lymphoma: Diagnosis and Treatment.

    Science.gov (United States)

    Ansell, Stephen M

    2015-11-01

    Hodgkin lymphoma is a rare B-cell malignant neoplasm affecting approximately 9000 new patients annually. This disease represents approximately 11% of all lymphomas seen in the United States and comprises 2 discrete disease entities--classical Hodgkin lymphoma and nodular lymphocyte-predominant Hodgkin lymphoma. Within the subcategorization of classical Hodgkin lymphoma are defined subgroups: nodular sclerosis, mixed cellularity, lymphocyte depletion, and lymphocyte-rich Hodgkin lymphoma. Staging of this disease is essential for the choice of optimal therapy. Prognostic models to identify patients at high or low risk for recurrence have been developed, and these models, along with positron emission tomography, are used to provide optimal therapy. The initial treatment for patients with Hodgkin lymphoma is based on the histologic characteristics of the disease, the stage at presentation, and the presence or absence of prognostic factors associated with poor outcome. Patients with early-stage Hodgkin lymphoma commonly receive combined-modality therapies that include abbreviated courses of chemotherapy followed by involved-field radiation treatment. In contrast, patients with advanced-stage Hodgkin lymphoma commonly receive a more prolonged course of combination chemotherapy, with radiation therapy used only in selected cases. For patients with relapse or refractory disease, salvage chemotherapy followed by high-dose treatment and an autologous stem cell transplant is the standard of care. For patients who are ineligible for this therapy or those in whom high-dose therapy and autologous stem cell transplant have failed, treatment with brentuximab vedotin is a standard approach. Additional options include palliative chemotherapy, immune checkpoint inhibitors, nonmyeloablative allogeneic stem cell transplant, or participation in a clinical trial testing novel agents.

  18. Salvage abdominal irradiation for refractory non-Hodgkin′s lymphoma

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    Akoum Riad

    2007-01-01

    Full Text Available Background: Abdominal irradiation, as a part of treatment, is often ignored in the management of refractory non-Hodgkin′s lymphoma (NHL. Objective: To evaluate the efficacy and the toxicity of this approach after failure of chemotherapy. Materials and Methods: 27 patients with intraabdominal lymphoma underwent salvage irradiation between 1982 and 2001. All patients were treated with a Cobalt-60 machine. The total dose administered to the abdomen was 18-20 Gy at the rate of 1.5-1.8 Gy per daily fraction, followed by a boost to gross disease up to 20 Gy. All patients had previously been heavily pretreated with chemotherapy. Fourteen patients, nine with follicular and five with diffuse lymphomas, had primary refractory tumors that had never achieved remission. Thirteen patients, six with follicular and seven with aggressive tumors, had refractory relapsed tumors after achieving one or more complete remissions. Results: The response rate was 77%. The median follow-up was 53 months. The 5-year and 10-year survival rates were 25 and 17%, respectively. The in-field and out-of-field recurrence rates were 22 and 33%, respectively. Survival rates were significantly better for patients with refractory relapse compared to those with primary refractory lymphoma (P < 0.01. There was no significant difference in terms of response, recurrence, or survival rates between follicular and aggressive types. Out-of-field recurrence occurred more frequently in initial stage III and IV disease. Toxic deaths occurred in three patients (11%. Conclusion: Salvage radiotherapy for refractory abdominal NHL is a feasible alternative for both follicular and diffuse subtypes and may provide significant palliation and prolongation of survival. It is less effective in patients with primary refractory NHL than in those with refractory relapsed NHL.

  19. Cell-free DNA in human follicular fluid as a biomarker of embryo quality.

    Science.gov (United States)

    Scalici, E; Traver, S; Molinari, N; Mullet, T; Monforte, M; Vintejoux, E; Hamamah, S

    2014-12-01

    Could cell-free DNA (cfDNA) quantification in individual human follicular fluid (FF) samples become a new non-invasive predictive biomarker for in vitro fertilization (IVF) outcomes? CfDNA level in human follicular fluid samples was significantly correlated with embryo quality and could be used as an innovative non-invasive biomarker to improve IVF outcomes. CfDNA fragments, resulting from apoptotic or necrotic events, are present in the bloodstream and their quantification is already used as a biomarker for gynaecological and pregnancy disorders. Follicular fluid is important for oocyte development and contains plasma components and factors secreted by granulosa cells during folliculogenesis. CfDNA presence in follicular fluid and its potential use as an IVF outcome biomarker have never been investigated. One hundred individual follicular fluid samples were collected from 43 female patients undergoing conventional IVF (n = 26) or ICSI (n = 17). CfDNA level was quantified in each individual follicular fluid sample. At oocyte collection day, follicles were aspirated individually. Only blood-free follicular fluid samples were included in the study. Follicle size was calculated based on the follicular fluid volume. Each corresponding cumulus-oocyte complex was isolated for IVF or ICSI procedures. Follicular fluid cfDNA was measured by quantitative PCR with ALU-specific primers. Human follicular fluid samples from individual follicles contain measurable amounts of cfDNA (mean ± SD, 1.62 ± 2.08 ng/µl). CfDNA level was significantly higher in small follicles (8-12 mm in diameter) than in large ones (>18 mm) (mean ± SD, 2.54 ± 0.78 ng/µl versus 0.71 ± 0.44 ng/µl, respectively, P = 0.007). Moreover, cfDNA concentration was significantly and negatively correlated with follicle size (r = -0.34; P = 0.003). A weak significant negative correlation between DNA integrity and 17β-estradiol level in follicular fluid samples at oocyte collection day was observed (r = -0

  20. [Plasmablastic lymphoma].

    Science.gov (United States)

    Fernández-Álvarez, Rubén; Sancho, Juan-Manuel; Ribera, Josep-María

    2016-11-04

    Plasmablastic lymphoma (PBL) is a rare and aggressive subtype of non-Hodgkin lymphoma that commonly occurs in human immunodeficiency virus (HIV)-positive individuals, and affects oral sites. Occasionally, it has been described in HIV-negative patients and involving non-oral sites. Pathologically, PBL is a high-grade B-cell lymphoma that displays the immunophenotype of a terminally differentiated B-lymphocyte with loss of B-cell markers (CD20) and expression of plasma-cell antigens. Epstein-Barr virus infection and MYC rearrangements are frequently observed. Treatment of PBL is challenging because of the lack of established treatment and poor outcomes, with median survival times shorter than one year. In this review, we discuss the clinical and epidemiologic spectrum of PBL as well as its distinct pathological features. Finally, we summarize the currently available approaches for the treatment of patients with PBL. Copyright © 2016 Elsevier España, S.L.U. All rights reserved.

  1. [Malignant Lymphoma of the Brain, and Dementia].

    Science.gov (United States)

    Mizutani, Saneyuki; Mizutani, Tomohiko

    2016-04-01

    A differential diagnosis of acute and subacute progressive dementias includes malignant lymphoma of the brain. We reviewed primary central nervous system lymphoma (PCNSL), intravascular lymphomatosis (IVL), lymphomatosis cerebri, and the relapse and invasion of systemic lymphomas. PCNSL is confined to the central nervous system; the infiltration and compression by the lymphoma result in adverse neurological symptoms. IVL is a rare form of malignant lymphoma that is characterized by the proliferation of primarily B-cell type lymphoma cells within the blood vessels of various organs. This causes ischemia and results in the associated neurological symptoms. Medical history and neuroimaging studies provide crucial informations to distinguish the lymphomas from other diseases that cause dementia, such an Alzheimer's disease. MRI imaging of the brain using contrast agent, and the biopsy of diseased tissues are essential for the diagnosis of the lymphomas. A histopathological examination is the most effective way to diagnose malignant lymphomas of the brain. Presently, the treatment of choice for PCNSL is the intravenous administration of high dose methotrexate with and without radiation therapy. Futhermore, Rituximab-containing chemotherapy has proved to greatly improve the prognosis of IVL. A better outcome can be achieved with the earlier diagnosis and treatment of the malignant lymphoma of the brain.

  2. Lymphoma of the eyelid

    DEFF Research Database (Denmark)

    Svendsen, Frederik H; Heegaard, Steffen

    2017-01-01

    Lymphoma of the eyelid constitutes 5% of ocular adnexal lymphoma. In previously published cases, 56% of lymphomas of the eyelid are of B-cell origin and 44% are of T-cell origin. The most frequent B-cell lymphomas are extranodal marginal zone lymphoma (27 cases-14%) and diffuse large B-cell lymph......Lymphoma of the eyelid constitutes 5% of ocular adnexal lymphoma. In previously published cases, 56% of lymphomas of the eyelid are of B-cell origin and 44% are of T-cell origin. The most frequent B-cell lymphomas are extranodal marginal zone lymphoma (27 cases-14%) and diffuse large B...... chemotherapy with or without adjuvant treatment is the treatment of choice for high-grade or disseminated lymphomas. The majority of subtypes, especially low-grade subtypes, have a good prognosis with few recurrences or progression. Some subtypes, including mycosis fungoides, have a poorer prognosis...

  3. Intracranial manifestations of malignant lymphomas

    Energy Technology Data Exchange (ETDEWEB)

    Galanski, M.; Fahrendorf, G.; Urbanitz, D.; Beckmann, A.; Elger, C.

    1985-06-01

    Approximately 10% of patients with malignant lymphoma will show neurological symptoms at some time during the course of their illness. In non-Hodgkin lymphoma, CNS involvement is more frequent than in Hodgkin's disease. Diffuse histiocytic and poorly differentiated lymphomas, bone marrow involvement, advanced tumor stage and hematogenous spread are particular risk factors. Invasion of the spinal canal is the most common type of CNS involvement. Intracranial lesions, which are comparatively rare, may present as intracerebral metastases, epi- or subdural masses or focal or diffuse leptomeningeal disease. Lymphomatous leptomeningitis usually cannot be demonstrated by CT. On the other hand, dural and cerebral parenchymal lesions are sometimes highly characteristic of lymphoma as a result of their features and location.

  4. Follicular Dowling Degos disease: A rare variant of an evolving dermatosis

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    Saurabh Singh

    2013-01-01

    Full Text Available Dowling Degos disease is a rare, reticulate pigmentary disorder with variable phenotypic expression that manifests as hyperpigmented macules and reticulate pigmentary anomaly of the flexures. Many variants of this condition and its overlap with other reticulate pigmentary disorders have been reported in the literature. We present here two cases of DDD with follicular localization, both clinically and histologically. It was associated with ichthyosis vulgaris in one case. Follicular DDD is an uncommon variant of this evolving dermatosis. Our report supports the possible role for disordered follicular keratinisation in its pathogenesis.

  5. Extending the Impact of RAC1b Overexpression to Follicular Thyroid Carcinomas

    Science.gov (United States)

    Faria, Márcia; Capinha, Liliana; Simões-Pereira, Joana; Bugalho, Maria João; Silva, Ana Luísa

    2016-01-01

    RAC1b is a hyperactive variant of the small GTPase RAC1 known to be a relevant molecular player in different cancers. Previous studies from our group lead to the evidence that its overexpression in papillary thyroid carcinoma (PTC) is associated with an unfavorable prognosis. In the present study, we intended to extend the analysis of RAC1b expression to thyroid follicular neoplasms and to seek for clinical correlations. RAC1b expression levels were determined by RT-qPCR in thyroid follicular tumor samples comprising 23 follicular thyroid carcinomas (FTCs) and 33 follicular thyroid adenomas (FTAs). RAC1b was found to be overexpressed in 33% of carcinomas while no RAC1b overexpression was documented among follicular adenomas. Patients with a diagnosis of FTC were divided into two groups based on longitudinal evolution and final outcome. RAC1b overexpression was significantly associated with both the presence of distant metastases (P = 0.01) and poorer clinical outcome (P = 0.01) suggesting that, similarly to that previously found in PTCs, RAC1b overexpression in FTCs is also associated with worse outcomes. Furthermore, the absence of RAC1b overexpression in follicular adenomas hints its potential as a molecular marker likely to contribute, in conjunction with other putative markers, to the preoperative differential diagnosis of thyroid follicular lesions. PMID:27127508

  6. Extending the Impact of RAC1b Overexpression to Follicular Thyroid Carcinomas

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    Márcia Faria

    2016-01-01

    Full Text Available RAC1b is a hyperactive variant of the small GTPase RAC1 known to be a relevant molecular player in different cancers. Previous studies from our group lead to the evidence that its overexpression in papillary thyroid carcinoma (PTC is associated with an unfavorable prognosis. In the present study, we intended to extend the analysis of RAC1b expression to thyroid follicular neoplasms and to seek for clinical correlations. RAC1b expression levels were determined by RT-qPCR in thyroid follicular tumor samples comprising 23 follicular thyroid carcinomas (FTCs and 33 follicular thyroid adenomas (FTAs. RAC1b was found to be overexpressed in 33% of carcinomas while no RAC1b overexpression was documented among follicular adenomas. Patients with a diagnosis of FTC were divided into two groups based on longitudinal evolution and final outcome. RAC1b overexpression was significantly associated with both the presence of distant metastases (P = 0.01 and poorer clinical outcome (P = 0.01 suggesting that, similarly to that previously found in PTCs, RAC1b overexpression in FTCs is also associated with worse outcomes. Furthermore, the absence of RAC1b overexpression in follicular adenomas hints its potential as a molecular marker likely to contribute, in conjunction with other putative markers, to the preoperative differential diagnosis of thyroid follicular lesions.

  7. Chronic Intussusception Caused by Diffuse Large B-Cell Lymphoma in a 6-Year-Old Girl Presenting with Abdominal Pain and Constipation for 2 Months.

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    Choi, Sun-Hee; Han, Sang-Ah; Won, Kyu Yeoun

    2016-02-01

    The classical triad of abdominal pain, vomiting, and bloody stool is absent in chronic intussusception for more than 2 weeks. Here, we report a 6-year-old female with recurrent abdominal pain for 2 months. Ultrasonography of the abdomen revealed an ileocolic-type intussusception. The lesion accompanying the tight fibrous adhesion was treated by resection and ileocolic anastomosis. It was diagnosed as intussusception with diffuse large B-cell lymphoma. A high index of suspicion for abdominal pain in children should result in the correct diagnosis and appropriate management.

  8. Peripartum Primary Prophylaxis Inferior Vena Cava Filter Placement in a Patient with Stage IV B-Cell Lymphoma Presenting with a Pathologic Femur Fracture

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    David M. Sherer

    2015-10-01

    Full Text Available Background  -Pulmonary embolus (PE remains a leading etiology of maternal mortality in the developed world. Increasing utilization of retrievable inferior vena cava (IVC filter placement currently includes pregnant patients. Case - A 22-year-old woman at 27 weeks' gestation was diagnosed with Stage IV high-grade malignant B cell lymphoma following pathologic femur fracture. Significant risk factors for PE led to placement of primary prophylaxis IVC filter before cesarean delivery, open reduction and internal fixation of the fractured femur, and chemotherapy. Conclusion - This case supports that primary prophylaxis placement of IVC filters in highly selected pregnant patients may assist in decreasing PE-associated maternal mortality.

  9. A gene panel, including LRP12, is frequently hypermethylated in major types of B-cell lymphoma.

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    Nicole Bethge

    Full Text Available Epigenetic modifications and DNA methylation in particular, have been recognized as important mechanisms to alter gene expression in malignant cells. Here, we identified candidate genes which were upregulated after an epigenetic treatment of B-cell lymphoma cell lines (Burkitt's lymphoma, BL; Follicular lymphoma, FL; Diffuse large B-cell lymphoma, DLBCL activated B-cell like, ABC; and germinal center like, GCB and simultaneously expressed at low levels in samples from lymphoma patients. Qualitative methylation analysis of 24 candidate genes in cell lines revealed five methylated genes (BMP7, BMPER, CDH1, DUSP4 and LRP12, which were further subjected to quantitative methylation analysis in clinical samples from 59 lymphoma patients (BL, FL, DLBCL ABC and GCB; and primary mediastinal B-cell lymphoma, PMBL. The genes LRP12 and CDH1 showed the highest methylation frequencies (94% and 92%, respectively. BMPER (58%, DUSP4 (32% and BMP7 (22%, were also frequently methylated in patient samples. Importantly, all gene promoters were unmethylated in various control samples (CD19+ peripheral blood B cells, peripheral blood mononuclear cells and tonsils as well as in follicular hyperplasia samples, underscoring a high specificity. The combination of LRP12 and CDH1 methylation could successfully discriminate between the vast majority of the lymphoma and control samples, emphasized by receiver operating characteristic analysis with a c-statistic of 0.999. These two genes represent promising epigenetic markers which may be suitable for monitoring of B-cell lymphoma.

  10. Lymphoma and metastatic breast cancer presenting with palpable axillary and inguinal lymphadenopathy in a 40-year-old man with rheumatoid arthritis on anti-tumor necrosis factor α therapy: a case report

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    Datta Gourab

    2013-01-01

    Full Text Available Abstract Introduction We present the case of a 40-year-old man with severe rheumatoid arthritis being treated with high-dose anti-tumor necrosis factor α therapy (adalimumab, who developed simultaneous lymphoma and breast cancer with lymph node metastases. We describe strategies for investigations and management of this presentation. Case presentation A 40-year-old Caucasian man with severe rheumatoid arthritis being treated with high-dose adalimumab presented to our facility with a swollen leg and palpable left groin and left axillary lumps and a left nipple lesion. Left lower limb ultrasound, computed tomography and positron emission tomography scans showed extensive lymphadenopathy. Core biopsies of the left groin, axilla and nipple lesion showed this to be concurrent diffuse B-cell lymphoma and locally metastatic invasive ductal carcinoma of the breast. He underwent a left mastectomy with axillary clearance, and adjuvant fluorouracil, epirubicin and cyclophosphamide chemotherapy with rituximab, and the adalimumab was stopped. Conclusions The findings from our patient’s case should increase awareness that patients with severe rheumatoid arthritis, especially if they are on high-dose biological treatments, have the potential to develop lymphoma, which in turn increases the risk of developing other primary tumors, so that in rare cases a patient may have concurrent tumors. Assessment and management of these patients is challenging and should include computed tomography scans of the of neck, thorax, abdomen and pelvis, including a fludeoxyglucose positron emission tomography/computed tomography scan, bone marrow testing and appropriate core biopsies and discussion at multidisciplinary team meetings about treatment of the separate tumors in the presence of hematologists, oncologists, surgeons and rheumatologists.

  11. Clinicopathological profile of gastrointestinal lymphomas in Kashmir

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    Mehnaaz Sultan Khuroo

    2016-01-01

    Full Text Available Background: The histological categorization of lymphoma has been a source of controversy for many years for both clinicians and pathologists. Clinicopathologic information of gastrointestinal lymphomas in Indian subcontinent is lacking. We studied histopathological spectrum of Primary Gastrointestinal Lymphomas (PGIL and attempted to classify the G.I. lymphomas based on the recent WHO classification in to major histological types and immunological categories. Material and Methods: This study was done to evaluate the clinicopathological pattern of 100 cases with a histopathological diagnosis of primary gastrointestinal lymphoma at a tertiary care hospital. All patients of primary gastrointestinal lymphomas were included with the help of medical records over a 11-years period that is, January 2005 to December 2015. Results: The study included 100 cases (60 males, 40 females; mean age 51.43 years; age range 4.5-90 years . The disease involved stomach in 82 (82%, small intestine in 8 (8%, large bowel and rectum in 8 (8%, gall bladder in 1 (1% and oesophagus in 1 (1%. 82 (82% of the 100 cases were Diffuse Large B cell lymphomas; 12 (12% were Extra Nodal Marginal Zone Lymphomas (ENMZL of MALT type 2 (2% IPSID 2 (2% of Mantle cell lymphoma morphology, 1 (1% Burkitt's and 1(1% enteropathy associated T cell lymphoma. The commonest presenting symptom was abdominal pain. 99 (99% of 100 tumours were classified as B-cell lymphomas immunohistochemically and majority exhibited monoclonal light chain restriction on kappa/lambda staining. In addition; Burkitt's lymphoma showed positivity for CD 10. One tumour (1% showed positivity for T-cell markers. The data demonstrated that primary GI NHL is more common among males, mainly in their fifth decade. Abdominal pain is the most common presenting symptom, with stomach being the most commonly involved site. Diffuse large cell lymphoma is the most frequent histologic subtype, followed by extranodal marginal-zone B

  12. Primary malignant lymphoma of the parotid gland

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    Sudha H Metikurke

    2012-01-01

    Full Text Available Lymphoma of the salivary gland accounts for 5% of cases of extranodal lymphoma and 10% of malignant salivary gland tumors. Most primary salivary gland lymphomas are B marginal zone lymphomas arising on a background of sialadenitis associated with an autoimmune disorder such as Sjorgen′s syndrome. This report describes a case of primary B-cell lymphoma arising in the parotid gland in a middle-aged female, which was not associated with an autoimmune disorder. Immunohistochemistry studies confirmed the clonal B-cell nature of the tumor. This case highlights the fact that B-cell lymphoma in the salivary gland can go unrecognized due to its non-specific symptoms and requires immunohistochemistry studies for confirmation. We present this case for its rarity.

  13. Primary thyroid lymphoma: A rare disease

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    Deepti Verma

    2014-01-01

    Full Text Available Primary thyroid lymphomas are rare neoplasms comprising of 1-5% of thyroid malignancies. These are predominantly B-cell in origin. Here, we report a case of 60 years lady, a known case of lymphocytic thyroiditis, diagnosed as thyroid lymphoma (diffuse large B-cell on fine needle aspiration and confirmed histopathogically and immunohistochemically. She presented with a sudden increase in thyroid swelling. Fine needle aspiration performed showed highly cellular smears comprising predominantly of the monomorphic population of medium to large sized lymphoid cells with high nuclear/cytoplasmic ratio and scant cytoplasm. A possibility of thyroid lymphoma possibly diffuse large B-cell lymphoma was suggested which was later confirmed on biopsy. Fine needle aspiration provides an easy mode for diagnosing large cell lymphoma like diffuse large B-cell. Hence, an early diagnosis is possible for a timely intervention. Also, cases of lymphocytic thyroiditis should be regularly followed for the development of lymphoma.

  14. Composite Lymphoma: Opposite Ends of Spectrum Meet

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    Khan, Uqba; Hadid, Tarik; Ibrar, Warda; Sano, Dahlia; Al-Katib, Ayad

    2017-01-01

    An 18-year-old African-American female presented with an episode of syncope. Initial investigations revealed large lung mass with invasion into right atrium along with lesions in kidneys and liver. Patient also developed superior vena cava syndrome due to lung mass. Biopsy of lung mass revealed diagnosis of composite lymphoma with involvement by primary mediastinal B-cell lymphoma (PMBCL) and classical Hodgkin lymphoma. Patient was started on dose-adjusted etoposide, prednisone, vincristine, cyclophosphamide, doxorubicin and rituximab (EPOCH-R) with complete response to treatment. This case represents an extremely rare type of aggressive lymphoma and can guide clinicians in managing such cases since there are no standard guidelines for treatment. To the best of our knowledge, this is the first reported case of composite lymphoma of PMBCL and classical Hodgkin lymphoma successfully treated with dose-adjusted EPOCH-R regimen.

  15. IgG4-related disease simulating Hodgkin lymphoma in a child

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    D. Eric Ewing, MD

    2016-06-01

    Full Text Available Immunoglobulin (Ig G4-related disease is a recently described syndrome characterized by mass forming lymphoplasmacytic tissue infiltration and elevated serum IgG4 concentrations usually affecting middle-aged or older individuals. Lymphadenopathy is frequently observed and is sometimes the first or only manifestation of the disease. We report a case of IgG4-related disease mimicking Hodgkin lymphoma in a 13-year-old girl. The patient presented with progressive unilateral cervical lymphadenopathy of several months duration. Biopsy showed follicular hyperplasia with progressive transformation of germinal centers. Interfollicular areas were expanded by small lymphocytes, histiocytes, eosinophils and fibrosis with occasional CD30 positive cells initially concerning for interfollicular Hodgkin lymphoma. Immunohistochemical analysis revealed an intrafollicular plasmacytosis with an IgG4-positive/IgG-positive plasma cell ratio of 50% supporting a diagnosis of IgG4-related lymphadenopathy, progressively transformed germinal centers type. Laboratory studies were supportive with elevated serum IgG4 (178 mg/dL and IgE (30.40 kU/L levels along with an elevated serum IgG4/IgG ratio (0.16. Very few cases of IgG4-related disease have been described in children. Within this age group, there is considerable clinical overlap between IgG4-related disease associated lymphadenopathy and Hodgkin lymphoma. In addition, lymphadenopathy secondary to IgG4-related disease demonstrates substantial histologic diversity with the potential to simulate the inflammatory background and fibrosis of Hodgkin lymphoma. The importance of accurate diagnosis is underscored by the prognostic implications considering the marked response of the syndrome to steroid therapy. In addition, appropriate follow up is critical to monitor for relapse and additional organ involvement.

  16. Perfluoroalkyl acid contamination of follicular fluid and its consequence for in vitro oocyte developmental competence.

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    Petro, Evi M L; D'Hollander, Wendy; Covaci, Adrian; Bervoets, Lieven; Fransen, Erik; De Neubourg, Diane; De Pauw, Ingrid; Leroy, Jo L M R; Jorssen, Ellen P A; Bols, Peter E J

    2014-10-15

    Perfluoroalkyl acids (PFAAs) have been shown to induce negative effects in laboratory animals and in vitro experiments. Also, PFAAs have been detected in human tissues and body fluids. The ovarian follicle constitutes a fragile micro-environment where interactions between hormones, growth factors, the oocyte and surrounding somatic cells are essential to generate a fully competent oocyte. In vitro experiments suggest that PFAAs can influence this balance, but very scarce in vivo data are available to confirm this assumption. In fact, the potential PFAA-presence in the follicular micro-environment is currently unknown. Therefore, we investigated if PFAAs are present in human follicular fluid and if their presence could be a risk factor for in vivo exposed developing oocytes. Furthermore, we compared the PFAA-distribution within serum and follicular fluid. PFAAs were analyzed by LC/MS in follicular fluid (n=38) and serum (n=20) samples from women undergoing assisted reproductive technologies (ARTs). Statistical models were used to investigate PFAA-distribution in both body fluids, to compare this behavior with the distribution of lipophilic organic pollutants and to explore the relationship between patient characteristics, ART-results and follicular fluid contamination. Perfluorooctane sulfonate (PFOS) was the PFAA found in the highest concentration in follicular fluid [7.5 (0.1-30.4) ng/mL] and serum [7.6 (2.8-12.5) ng/mL]. A new variable, Principal Component 1, representing the overall PFAA-contamination of the follicular fluid samples, was associated with a higher fertilization rate (porganic pollutants as explanatory variables. To conclude, overall higher PFAA-contamination in the follicular micro-environment was associated with a higher chance of an oocyte to develop into a high quality embryo. Also, PFAAs have different distribution patterns between serum and follicular fluid compared to the lipophilic organic pollutants. Further research is of course crucial

  17. Autoimmune hemolytic anaemia in Hodgkin's lymphoma.

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    Shah, Mihir B; Nanjapp, Veena; Devaraj, H S; Sindhu, K S

    2013-07-01

    Autoimmune hemolytic anaemia is a rare presentation of Hodgkin's lymphoma though its association with Non- Hodgkin's lymphoma is well known. It is usually detected at the time of diagnosis when it accompanies Hodgkin's and rarely precedes it. It is a warm immune hemolytic anemia which is responsive to steroids and rituximab. We hereby report a case of advanced Hodgkin's disease who presented as AIHA.

  18. Plasma cells negatively regulate the follicular helper T cell program

    OpenAIRE

    2010-01-01

    B lymphocytes differentiate into antibody-secreting cells under the antigen-specific control of follicular helper T (TFH) cells. Here, we demonstrate that isotype-switched plasma cells expressed MHCII, CD80 and CD86 and intracellular machinery required for antigen presentation. Antigen-specific plasma cells could access, process and present sufficient antigen in vivo to induce multiple TH cell functions. Importantly, antigen-primed plasma cells failed to induce interleukin 21 or Bcl-6 in naïv...

  19. Primary non-Hodgkin's lymphomas of the female breast.

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    Giardini, R; Piccolo, C; Rilke, F

    1992-02-01

    The charts of 35 women with primary malignant non-Hodgkin's lymphomas (NHL) of the breast were retrieved from the files of the Istituto Nazionale Tumori, Milan, over a 30-year period (1957 to 1986). These cases represented 0.1% of the more than 25,000 primary malignant tumors of the breast treated during the same period. The median age of these patients was 57 years (range, 28 to 81 years). In most cases, the clinical diagnosis was carcinoma. The tumors were either Stage IE(48%) or IIE(52%) at presentation, and only two patients had B symptoms. The right breast was involved in 17 patients, the left breast in 14, and both breasts in two. According to the updated Kiel classification and the Working Formulation (WF) for Clinical Usage, three cases were lymphoplasmacytoid (immunocytoma) NHL (WF, A); three, centroblastic-centrocytic, follicular NHL (WF, B); four, centroblastic-centrocytic, diffuse NHL (WF, F); 17 centroblastic NHL (WF, G); three immunoblastic NHL (WF, H); two B-lymphoblastic NHL (WF, I); and one, a Burkitt-like NHL (WF, J). Treatment consisted either of a combination of surgery, radiation therapy, and chemotherapy or radiation therapy and chemotherapy. The follow-up period for 32 patients ranged from 6 to 161 months (mean, 45 months); 17 patients died of their disease. The prognosis appeared to be related to the histologic type and stage of the disease. Median survival periods were 63, 52, 42, and 47 months for centroblastic-centrocytic follicular, centroblastic-centrocytic diffuse, centroblastic, and immunoblastic NHL, respectively. The overall 5-year survival rate was 43%; the 5-year survival rate and the probability of freedom from progression at 5 years were, respectively, 61% and 50% for Stage I and 27% and 26% for Stage II disease.

  20. Treatment of thyroid follicular carcinoma.

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    Ríos, Antonio; Rodríguez, José M; Parrilla, Pascual

    2015-12-01

    Differentiated thyroid carcinoma includes 2 different tumor types, papillary (PC) and follicular carcinoma (FC), and although similar, their prognosis is different. FC is uncommon, and this has led to it often being analyzed together with PC, and therefore the true reality of this tumor is difficult to know. As a result, the diagnostic and therapeutic management and the prognostic factors in differentiated carcinoma are more predictive of PC than FC. In this review we analyze the current state of many of the therapeutic aspects of this pathology. The best surgical technique and the usefulness of associated lymphadenectomy is also analyzed. Regarding post-surgical ablation with 131I, the indications, doses and usefulness are discussed. For the remaining therapies we analyze the few indications for radiotherapy and chemotherapy, and of new drugs such as tyrosine kinase inhibitors.